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Blut und Blutkrankheiten pp 222–352Cite as

Das v. Willebrand-Jürgens-Syndrom

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Part of the book series: Handbuch der Inneren Medizin ((INNEREN 2,volume 2 / 9))

Zusammenfassung

Beim v. Willebrand-Jürgens-Syndrom (vWJS) handelt es sich um eine in den meisten Fällen hereditäre, autosomal dominant oder rezessiv vererbte, selten aber auch erworbene hämorrhagische Diathese mit einer quantitativen und/oder qualitativen Störung des als v. Willebrand-Faktor (VIII:vWF) bezeichneten großmolekularen Anteils des Faktor-VIII/v. Willebrand-Faktor-Komplexes.

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Literatur

  • Abildgaard CF, Simone JV, Honig GR, Forman EN, Johnson CA, Seeler RA (1968) Von Willebrand’s disease. A comparative study of diagnostic tests. J Pediatr 73: 355

    Google Scholar 

  • Abildgaard CF, Suzuki Z, Harrison J et al. (1980) Serial studies in von Willebrand’s disease: Variability versus “variants.” Blood 56: 712–716

    CAS  Google Scholar 

  • Achenbach W (1960) Angiohaemophilie. Ergebnisse Innere Med Kinderheilkunde 14: 68

    CAS  Google Scholar 

  • Adashi EY (1980) Lack of improvement in von Willebrand’s disease during pregnancy. N Engl J Med 303: 1178 (letter)

    Google Scholar 

  • Adelman S, Monto RW, Ponka JL (1972) Surgery in von Willebrand’s disease. Arch Surg 105: 204

    PubMed  CAS  Google Scholar 

  • Ahr DJ, Hoyer LW, O’Leary DS, Conrad ME (1977) von Willebrand’s disease and hemorrhagic teleangiectasia. Association of two complex disorders of hemostasis resulting in lifethreatening hemorrhage. Am J Med 62: 452

    Google Scholar 

  • Aihara M, Cooper HA, Wagner RH (1984) Platelet-collagen interactions: increase in rate of adhesion of fixed washed platelets by factor VHI-related antigen. Blood 63: 495–501

    PubMed  CAS  Google Scholar 

  • Alexander B, Goldstein R (1953) Dual hemostatic defect in pseudohemophilia. J Clin Invest 32: 551

    Google Scholar 

  • Allain JR, Cooper HA, Wagner RH, Brinkhous KM (1975) Platelets fixed with paraformaldehyde: A new reagent for assay of von Willebrand factor and platelet aggregating factor. J Lab Clin Med 85: 318–32

    Google Scholar 

  • Allain JP, Verroust F, Soulier JP (1980) In vitro and in vivo characterisation of factor VIII preparations. Vox Sang 38: 68–80

    PubMed  CAS  Google Scholar 

  • Alperin JB (1982) Estrogens and surgery in women with von Willebrand’s disease. Am J Med 73: 367

    PubMed  CAS  Google Scholar 

  • Ardaillou N, Girma JP, Meyer D, Lavergne JM, Shoa’i I, Larrieu MJ (1978) “Variants” of von Willebrand’s disease. Demonstration of a decreased antigenetic reactivity by immunoradiometric assay. Thromb Res 12: 817–830

    Google Scholar 

  • Armitage H, Rizza CR (1979) Two populations of factor VIII related antigen in a family with von Willebrand’s disease. Br J Haematol 41: 279–289

    PubMed  CAS  Google Scholar 

  • Aursness I, Veninga C (1979) Detachment of von Willebrand factor from blood platelets. Thromb Haemost 42: 805

    Google Scholar 

  • Bachman F (1980) Diagnostic approach to mild bleeding disorders. Semin Hematol 17: 292–30

    Google Scholar 

  • Barbui T, Rodeghiero F, Dini E (1977) The aspirin tolerance test in von Willebrand’s disease. Thromb Haemost 38: 510–513

    PubMed  CAS  Google Scholar 

  • Barlow GH, Martin SE, Marder VJ (1984) Sedimentation analysis of von Willebrand and factor VIII C protein using partition cells in the analytical ultracentrifuge. Blood 63: 940–943

    PubMed  CAS  Google Scholar 

  • Barrow EM, Graham JB (1964) von Willebrand’s disease. Progr Hematol 4: 203

    Google Scholar 

  • Barrow ES, Reisner HM, Graham JB (1979) The separation of Willebrand factor from factor VIII related antigen. Br J Haematol 42: 455–468

    PubMed  CAS  Google Scholar 

  • Barrowcliffe TW, Kemball-Cook G, Morris G et al. (1981) Factor VHI-related activities in therapeutic concentrates. J Lab Clin Med 97: 429–438

    PubMed  CAS  Google Scholar 

  • Baumgartner HR (1973) The role of blood flow in platelet adhesion, fibrin deposition, and formation of mural thrombi. Micro vase Res 5: 167–179

    CAS  Google Scholar 

  • Baumgartner HR, Tschopp TB, Meyer D (1980) Shear rate dependent inhibition of platelet adhesion and aggregation on collagenous surfaces by antibodies to human factor VIII/von Willebrand factor. Br J Haematol 44: 127–139

    PubMed  CAS  Google Scholar 

  • Bennett B, Ratnoff OD (1972) Changes in antihemophilic factor ( AHF, factor VIII) procoagulant activity and AHF-like antigen in normal pregnancy, and following exercise and pneumoencephalographie. J Lab Clin Med 80: 256

    Google Scholar 

  • Bennett B, Ratnoff OD, Levin J (1972) Immunologic studies in von Willebrand’s disease. Evidence that the antihemophilic factor ( AHF) produced after transfusions lacks an antigen associated with normal AHF and the inactive material produced by patients with classic hemophilia. J Clin Invest 51: 2597–2601

    Google Scholar 

  • Bennett E, Dormandy K (1966) Pool’s cryoprecipitate and exhausted plasma in the treatment of von Willebrand’s disease and factor XI deficiency. Lancet 11: 731–732

    Google Scholar 

  • Biggs R, Matthews JM (1963) The treatment of haemorrhage in von Willebrand’s disease and the blood level of factor VIII ( AHF ). Br J Haematol 9: 203

    Google Scholar 

  • Bird P, Rizza CR (1975) A method for detecting factor VIII clotting activity associated with factor VHI-related antigen in agarose gels. Br J Haematol 31: 5 — 12

    PubMed  CAS  Google Scholar 

  • Biggs R, Rizza CR (1978) The control of haemostasis and hemophilic patients. In: Biggs R (ed) The treatment of haemophilia A and B and von Willebrand’s disease. Blackwell Scientific Publ, Oxford, p 127

    Google Scholar 

  • Björlin G, Nilsson IM (1973) Tooth extractions in hemophiliacs after administration of a single dose of factor VIII or factor IX concentrate supplemented with AMCA. Oral Surg, Oral Medicine, Oral Pathology 36: 482–489

    Google Scholar 

  • Blatt PM, Brinkhous KM, Culp HR et al. (1976) Antihemophilic factor concentrate therapy in von Willebrand’s disease. Dissociation of bleeding time factor and ristocetincofactor activities. JAMA 236: 2770–277

    PubMed  CAS  Google Scholar 

  • Blättler W, Jacky E, Müller M, Graf M (1979) Der Einfluß von l-Desamino-8-D-Arginine-Vasopressin ( DD A VP) auf die Blutgerinnung bei Patienten mit Haemophilie A und gesunden Männern. Schweiz Med Wochenschr 109: 1367

    Google Scholar 

  • Blättler W, Graf M, Jacky E, Müller M (1980) The kinetics of factor VIII upon l-deamino-D-arginine-vasopressin (DD A VP) in hemophiliacs and normal men. In: Sutor AH (ed) DD A VP in bleeding disorders. 1st Int Symp on DDAVP in bleeding disorders, Münster 79. Schattauer, Stuttgart New York, p 52

    Google Scholar 

  • Blombäck B, Blombäck M (1956) Purification of human and bovine fibrinogen. Ar Kir Kemi 10: 415

    Google Scholar 

  • Blomback M, Jorpes J, Nilsson IM (1963) von Willebrand’s disease. Am J Med 34: 236–241

    Google Scholar 

  • Bloom AL (1977) Physiology of factor VIII. In: Poller L (ed) Recent advances in blood coagulation, 2nd edn. Churchill Livingstone, Edinburgh, pp 141–181

    Google Scholar 

  • Bloom AL (1979) The biosynthesis of factor VIII. Clin Haematol 8: 53–77

    PubMed  CAS  Google Scholar 

  • Bloom AL (1980) The von Willebrand Syndrome. Semin Hematol XVII: 215–227

    Google Scholar 

  • Bloom AL, Peake IR (1977) Molecular genetics of factor VIII and its disorders. Semin Hematol 14: 319–33

    PubMed  CAS  Google Scholar 

  • Bloom AL, Peake IR (1979) Apparent “Dominant” and “Recessive” inheritance of von Willebrand’s disease within the same kindreds. Possible biochemical mechanisms. Thromb Res 15: 505–512

    Google Scholar 

  • Bloom AL, Giddings JC, Wilks CJ ( 1973 a) Factor VIII on the vascular intima: Possible importance in haemostasis and thrombosis. Nature 241: 217–219

    Google Scholar 

  • Bloom AL, Peake IR, Giddings JC (1973 b) The presence and reactions of high and lower molecular weight procoagulant factor VIII, in the plasma of patients with von Willebrand’s disease after treatment; significance for a structural hypothesis for factor VIII. Thromb Res 3: 389

    Google Scholar 

  • Bloom AL, Peake IR, Giddings JD et al. (1976) Endothelial cells and factor Vlll-related protein. Lancet 1: 46

    PubMed  CAS  Google Scholar 

  • Bloom AL, Peake IR, Furlong RA et al. (1979) High potency factor VIII concentrate: More effective than cryoprecipitate in a patient with von Willebrand’s disease and inhibitor. Thromb Res 16: 847–85

    PubMed  CAS  Google Scholar 

  • Bolhuis PA, Sakariassen KS, Sixma J J (1979) Adhesion of blood platelets to human arterial subendothelium: Role of factor VIII-von Willebrand factor. Haemostasis 8: 312–323

    Google Scholar 

  • Bolhuis PA, Sakariassen KS, Sander HJ et al. (1981) Binding of factor VIII-von Willebrand factor to human arterial subendothelium precedes increased platelet adhesion and enhances platelet spreading. J Lab Clin Med 97: 568

    PubMed  CAS  Google Scholar 

  • Borchgrevink CF (1960) A method for measuring platelet adhesiveness in vivo. Acta Med Scand 168: 157

    Google Scholar 

  • Borchgrevink CF, Waaler BA (1958) The secondary bleeding time. A new method for the differentiation of hemorrhagic diseases. Acta Med Scand CLXII: 361–374

    Google Scholar 

  • Borchgrevink CF, Egeberg O, Godal HC, Hjort PF (1963) The effect of plasma and Cohn’s fraction I on the Duke and Ivy bleeding times in von Willebrand’s disease. Acta Med Scand 173: 235

    PubMed  CAS  Google Scholar 

  • Born GVR (1962) Aggregation of blood platelets by adenosine diphosphate and its reversal. Nature 194: 927

    PubMed  CAS  Google Scholar 

  • Böttcher D, Hasler K, Winckelmann G, Sutor AH, Mair D (1978) Zur Diagnostik des von Willebrand Jürgens Syndroms. Med Klin 73: 833–838

    PubMed  Google Scholar 

  • Böttcher D, Hasler K, Schmidt B, Winckelmann G (1980) Diagnostische Wertigkeit der Ristocetin-Cofaktor-Bestimmung. In: Voss H von, Göbel U (Hrsg) Praktische Anwendung der Thrombozytenfunktionsdiagnostik. Thieme, Stuttgart, S 156

    Google Scholar 

  • Bouma BN, Wiegerinck Y, Sixma JJ et al. (1972) Immunological characterization of purified antihaemophilic factor A (factor VIII) which corrects abnormal platelet retention in von Willebrand’s disease. Nature 236: 104 - 106

    CAS  Google Scholar 

  • Bowie EJW, Owen CA Jr, Thompson JH Jr, Didisheim P (1969) Platelet adhesiveness in von Willebrand’s disease. Am J Clin Pathol 52: 69–77

    PubMed  CAS  Google Scholar 

  • Bowie EJW, Owen CA Jr, Zollman PE (1973) Tests of hemostasis in swine: Normal values and values in pigs affected with von Willebrand’s disease. Am J Vet Res 34: 1405–1407

    Google Scholar 

  • Bowie EJW, Fass DN, Olson JD, Owen CA Jr (1974) Transfusion and autotransfusion of plasma in von Willebrand’s disease. Thromb Res 5: 479

    Google Scholar 

  • Bowie EJW, Fass DN, Olson JD, Owen CA (1976) The spectrum of von Willebrand’s disease revisited. Mayo Clin Proc 51: 35

    PubMed  CAS  Google Scholar 

  • Bowie EJW, Fass DN, Owen CA Jr (1980) Hemostatic effect of transfused Willebrand factor in porcine von Willebrand’s disease. Haemostasis 9: 352–365

    PubMed  CAS  Google Scholar 

  • Bowie EJW, Fass DN, Katzmann JA (1981) The effect of antiporcine Willebrand factor monoclonal antibodies on the bleeding time. Thromb Haemost 46: 166 (abstract)

    Google Scholar 

  • Brinkhous KM, Read MS (1978) Preservation of platelet receptors for platelet aggregating factor/von Willebrand factor by air drying freezing, or lyophilization: New stable platelet preparations for von Willebrand factor assays. Thromb Res 13: 591–597

    Google Scholar 

  • Brinkhous KM, Read MS (1980) Use of venom coagglutinin and lyophilized platelets in testing for plateletaggregating von Willebrand factor. Blood 55: 517–520

    PubMed  CAS  Google Scholar 

  • Brinkhous KM, Graham JE, Cooper HA, Allain JP, Wagner RH (1975) Assay of von Willebrand factor in von Willebrand’s disease and hemophilia: Use of a macroscopic platelet aggregation test. Thromb Res 6: 267–272

    Google Scholar 

  • Brinkhous KM, Barnes DS, Potter JY, Read MS (1981) Von Willebrand syndrome induced by a Bothrops venom factor: Bioassay for venom coagglutinin. Proc Natl Acad Sei 78: 3230–3234

    Google Scholar 

  • Brockway WJ, Fass DN (1977) The nature of the interaction between ristocetin-Willebrand factor and the factor VIII coagulant activity molecule. J Lab Clin Med 89: 1295–1305

    PubMed  CAS  Google Scholar 

  • Brody JI (1975) Prolonged bleeding times with factor IX and XI deficiency (von Willebrand Syndromes). Am J Med Sei 269: 19

    CAS  Google Scholar 

  • Brown JE, Baugh RF, Hougie C (1979) Effect of exercise on the factor VIII complex: A correlation of the von Willebrand antigen and factor VIII coagulant antigen increase. Thromb Res 15: 61–6

    Google Scholar 

  • Buchanan GR, Green DM, Handin RI (1977 a) Combined von Willebrand disease and Hageman factor deficiency. J Pediatr 90: 779

    Google Scholar 

  • Buchanan GR, Martin V, Levine PH, Scoon K, Handin RI (1977b) The effects of “antiplatelet” drugs on bleeding time and platelet aggregation in normal human subjects. Am J Clin Pathol 68: 355–35

    PubMed  CAS  Google Scholar 

  • Buchanan JC, Leavell BS (1956) Pseudohemophilia: Report of 13 men cases and statistical review of previously reported cases. Ann Intern Med 44: 241

    PubMed  CAS  Google Scholar 

  • Budde U, Brackmann HH, Etzel F (1980) Einsatz von DDAVP bei zahnärztlichen Eingriffen bei Patienten mit von Willebrand Syndrom und einem Patienten mit kombiniertem Herzmansky-Pudlak-Syndrom und leichtem von Willebrand Syndrom. In: Sutor AH (ed) DDAVP in bleeding disorders (1st Int Symp on DDAVP in bleeding disorders, Münster 1979 ). Schattauer, Stuttgart New York, p 148

    Google Scholar 

  • Budde U, Schäfer G, Müller N, Hammerstein U, Lüchters G (1982) Vorkommen milder Formen des von Willebrand Syndroms Typ I in der „Normalbevölkerung“. In: Loo J van de, Asbeck F (Hrsg) Hämostase, Thrombophilie und Arteriosklerose, (Berichtband 2. Kongreß für Thrombose und Hämostase). Schattauer, Stuttgart New York, S 505

    Google Scholar 

  • Budde U, Schäfer G, Müller N et al. (1984) Acquired von Willebrand’s disease in the myeloproliferative syndrome. Blood 64: 981–985

    PubMed  CAS  Google Scholar 

  • Caduff T, Straub P (1979) Die Verwendung gefrorener Thrombozyten für die Bestimmung des von Willebrand Faktors mit Ristocetin. Schweiz Med Wochenschr 109: 399

    PubMed  CAS  Google Scholar 

  • Caen J, Larrieu MJ, Meyer D (1969) Diagnostic et traitement de la Maladie de Willebrand. Rev Med Suisse Romande 89: 67

    PubMed  CAS  Google Scholar 

  • Caen JP, Nurden AT, Jeanneau C, Michel H, Tobelem G, Levy-Toledano S, Sultan Y, Valensi F, Bernard J (1976) Bernard-Soulier Syndrome: a new platelet glycoprotein abnormality. Its relationship with platelet adhesion to subendothelium and with the factor VIII/von Willebrand protein. J Lab Clin Med 87: 586–596

    Google Scholar 

  • Chediak JR, Telfer MC, Green D (1977) Platelet function and immunologic parameters in von Willebrand’s disease following cryoprecipitate and factor VIII concentrate infusion. Am J Med 62: 369

    PubMed  CAS  Google Scholar 

  • Chediak JR, Lambert E, Johnson EI, Telfer MC (1980) Combined severe factor XI deficiency and von Willebrand’s disease. Am J Clin Pathol 74: 108

    PubMed  CAS  Google Scholar 

  • Chesney C, Colmon RW, Pechet L (1974) A syndrome of platelet-release abnormality and mild hemophilia. Blood 43: 821

    PubMed  CAS  Google Scholar 

  • Clemetson KJ, Lüscher EF (1985) Glykoproteine der Plättchenmembran. Haemostaseologie, im Druck

    Google Scholar 

  • Cockburn CG, Beaufre-Apps RJ de, Wilson J, Hardisty RM (1981) Parallel destruction of factor VIII procoagulant activity and an 85,000-dalton protein in highly purified factor VIII/vWF. Thromb Res 21: 295–30

    PubMed  CAS  Google Scholar 

  • Cohen JA (1972) Multiple congenital anomalies: the association of seven defects including multiple exostoses, von Willebrand’s disease, and bilatoral winged scapula. Arch Intern Med 129: 972

    PubMed  CAS  Google Scholar 

  • Coller BS, Peerschke EI, Scudder LE, Sullivan CA (1983) Studies with a murine monoclonal antibody that abolishes Ristocetin-induced binding of von Willebrand factor to platelets: Additional evidence in support of von GPIb as a platelet receptor for von Willebrand factor. Blood 61: 99–110

    Google Scholar 

  • Conlon CL, Weinger RS, Cimo PL, Moak JL, Olson JD (1978) Teleangiectasia and von Willebrand’s disease in two families. Ann Intern Med 89: 921

    PubMed  CAS  Google Scholar 

  • Cooper HA, Mason RG, Brinkhous KM (1976) The platelet: Membrane and surface reactions. Ann Rev Physiol 38: 501

    Google Scholar 

  • Corder MP, Culp NW, Barrett O Jr (1973) Familial occurrence of von Willebrand’s disease, thrombocytopenia, and severe gastrointestinal bleeding. Am J Med Sei 265: 219

    CAS  Google Scholar 

  • Cornu P (1965) Maladie de Willebrand. Pathol Biol (Paris) 13: 546

    CAS  Google Scholar 

  • Cornu P, Larrieu MJ, Caen J, Bernard J (1961) Maladie de Willebrand. Etude clinique, genetique et biologique. Nouv Rev Fr Hematol 1: 231

    Google Scholar 

  • Cornu P, Larrieu MJ, Caen J, Bernard J (1963) Transfusion studies in von Willebrand’s disease: Effect on bleeding time and factor VIII. Br J Haematol 9: 189–202

    Google Scholar 

  • Counts RB (1975) Solid-phase immunoradiometric assay of factor VIII protein. Br J Haematol 31: 429–43

    PubMed  CAS  Google Scholar 

  • Counts RB, Paskell SL, Elgee SK (1978) Disulfide bonds and the quaternary structure of factor VIII/von Willebrand factor. J Clin Invest 62: 702–709

    PubMed  CAS  Google Scholar 

  • Cramer AD, Melaragno AJ, Phifer SJ, Hougie C (1976) Von Willebrand disease San Diego, a men variant. Lancet 11: 12–1

    Google Scholar 

  • Crefeld S van, Kloosterman GJ, Mochtar IA, Koppe JG (1962) Interchange between blood of mother and fetus in vascular hemophilia. Biol Neonat 4: 173

    Google Scholar 

  • Czapek EE, Deykin D, Salzman E et al. (1978) Intermediate syndrome of platelet dysfunction. Blood 52: 103–13

    PubMed  CAS  Google Scholar 

  • Davies BL, Furlong RA, Peake IR (1981) Studies on the relationship between factor VHI-related antigen (VIIIRAg) and factor VIII clotting antigen (VIIICAg) by immunoelectrophoresis and autoradiography using 125 J anti-VIIICAg. Thromb Res 22: 87–96

    CAS  Google Scholar 

  • Donati MB, de Gaetano G, Vermylen J (1973) Evidence that bovine factor VIII, not bovine fibrinogen aggregates human platelets. Thromb Res 2: 97–104

    Google Scholar 

  • Doucet-de Bruine MHM, Sixma JJ, Over J, Beeser-Visser NH (1978) Heterogeneity of human factor VIII binding to platelets in the presence of ristocetin. J Lab Clin Med 92: 96–107

    Google Scholar 

  • Dowling SV, Müntz RH, D’Souza S, Ekert H (1976) Platelet release abnormality associated with a variant of von Willebrand’s disease. Blood 47: 265

    PubMed  CAS  Google Scholar 

  • Dudley NE, Kernoff PBA, Gough MH (1971) Surgery in children with congenital disorders of blood coagulation. J Pediatr Surg 6: 689

    PubMed  CAS  Google Scholar 

  • Duke WW (1910) The relation of blood platelets to hemorrhagic disease. JAMA 55: 1185–1192

    Google Scholar 

  • Egberg N, Blombäck M (1976) On the characterization of acquired inhibitors to ristocetin induced platelet aggregation found in patients with von Willebrand’s disease. Thromb Res 9: 527–531

    PubMed  CAS  Google Scholar 

  • Egeberg O (1965) An inherited hemorrhagic trait with characteristics resembling both mild hemophilia of type A and von Willebrand’s disease. Scand J Clin Lab Invest [Suppl] 84: 25

    Google Scholar 

  • Ekert H, Chavin SI (1977) Changes in electrophoretic mobility of human factor VIII related antigen: Evidence for a subunit structure. Br J Haematol 36: 271–279

    Google Scholar 

  • Elston RC, Graham JB, Miller CH, Reisner HM, Bouma BN (1976) Probalistic classification of haemophila A carriers by discriminant analysis. Thromb Res 8: 683

    PubMed  CAS  Google Scholar 

  • Eriksson AW (1961) Eine neue Blutersippe mit von Willebrand Jürgens’sche Krankheit (erbliche Thrombopathie) auf Äland (Finnland). Acta Gen Med (Roma) 10: 157–180

    CAS  Google Scholar 

  • Esham RH, Skilling FC Jr, Dodson WH, Hammack WJ (1974) Hereditary hemorrhagic teleangiectasia and factor VIII deficiency. Arch Intern Med 134: 327

    PubMed  CAS  Google Scholar 

  • Evans RJ, Austen DEG (1977) Assay of ristocetin cofactor using fixed platelets and a platelet counting technique. Br J Haematol 37: 289–294

    PubMed  CAS  Google Scholar 

  • Eyster ME, Ballard JO, Prager D (1978) Comparison of factor VIII levels after adrenalin in classic hemophilia and von Willebrand’s disease (vWD). Thromb Haemostas 39: 657–662

    CAS  Google Scholar 

  • Fasching I, Niessner H (1982) Aufgrund welcher Ereignisse wurde die Diagnose eines vWJS gestellt. Haemophilie Symposion Hamburg

    Google Scholar 

  • Fass DN, Brockway WJ, Owen CA, Bowie EJW (1976) Factor VIII ( Willebrand) antigen and ristocetin-Willebrand factor in pigs with von Willebrand’s disease. Thromb Res 8: 319

    Google Scholar 

  • Fass DN, Knutson GJ, Bowie EJW (1978) Porcine Willebrand factor: A population of multimers. J Lab Clin Med 91: 307–320

    Google Scholar 

  • Fass DN, Bowie EJW, Owen CA Jr, Zollman PE (1979) Inheritance of porcine von Willebrand’s disease: study of a kindred of over 700 pigs. Blood 53: 712–719

    PubMed  CAS  Google Scholar 

  • Fass DN, Knutson GJ, Katzmann JA (1982) Monoclonal antibodies to porcine factor VIII coagulant and their use in the isolation of active coagulant protein. Blood 59: 594–600

    PubMed  CAS  Google Scholar 

  • Finlay TH, Marcus D, Kowalski D, Silber P (1981) Interaction of porcine von Willebrand factor (platelet aggregating factor) with human platelets. Biochim Biophys Acta 672: 79–88

    PubMed  CAS  Google Scholar 

  • Forbes CD, Prentice CRM (1973) Aggregation of human platelets by purified porcine and bovine antihaemophilic factor. Nature 241: 149–150

    CAS  Google Scholar 

  • Fuhge P, Braun K, Heimburger N (1982) Neues Reagenz für die Bestimmung des Ristocetin-Cofak-tors, Faktor VIIIR:Cof. In: Loo J van de, Asbeck F (Hrsg) Hämostase, Thrombophilie und Arteriosklerose, (Berichtband 2. Kongreß für Thrombose und Hämostase) Schattauer, Stuttgart New York, S 700

    Google Scholar 

  • Fujimoto T, Hawiger J (1982) Adenosine diphosphate induces binding of von Willebrand factor to human platelets. Nature 297: 154

    PubMed  CAS  Google Scholar 

  • Fujimoto T, Ohara S, Hawiger J (1982) Thrombin-induced exposure and prostacyclin inhibition of the receptor for factor VIII/von Willebrand factor on human platelets. J Clin Invest 69: 1212

    PubMed  CAS  Google Scholar 

  • Fukui H, Mikami S, Takase T, Fujimura Y, Nishino M, Yoshioka A (1980) Patterns of factor VIII related antigen on crossed immunoelectrophoresis and large pore Polyacrylamide gel-crossed immunoelectrophoresis in von Willebrand’s disease. Br J Haematol 46: 269

    PubMed  CAS  Google Scholar 

  • Fulcher CA, Zimmerman TS (1982) Characterization of the human factor VIII procoagulant protein with a heterologous precipitating antibody. Proc Natl Acad Sci USA 79: 1648–1652

    PubMed  CAS  Google Scholar 

  • Fulcher CA, Ruggeri ZM, Zimmerman TS (1983) Isoelectric focusing of human von Willebrand factor in Urea-agarose gels. Blood 61: 304–310

    PubMed  CAS  Google Scholar 

  • Furlan M, Beck EA (1977) Degradation of purified factor VIII by endogeneous contaminating enzymes. Thromb Res 10: 153–158

    PubMed  CAS  Google Scholar 

  • Fuster V, Bowie EJW (1978) The von Willebrand pigs as a model for atherosclerosis research. Thromb Haemostas 39: 322–327

    CAS  Google Scholar 

  • Fuster V, Bowie EJW, Lewis JC (1978) Resistance to arteriosclerosis in pigs with von Wille-brand’s disease. Spontaneous and high cholesterol diet-induced arteriosclerosis. J Clin Invest 61: 722–73

    Google Scholar 

  • Gader AMA, Costa J da, Cash JD (1973) A new vasopressin analogue and fibrinolysis. Lancet 11: 1417–141

    Google Scholar 

  • Gaetano G de, Donati MB, Vermylen J (1974) Evidence that human platelet aggregating activity in porcine plasma is a property of von Willebrand factor. Thromb Diathes Haemorrh 32: 549–55

    CAS  Google Scholar 

  • Garcia VV, Silva JA, Borrasca AL (1982) Response of factor VIII/von Willebrand factor to intranasal DDAVP in healthy subjects and mild haemophiliacs (with observations in patients with combined deficiency of factor V and VIII). Thromb Haemostas 48: 91–93

    CAS  Google Scholar 

  • Gastaldi G, Rasore-Quartino A, Galletti A, Campanella A, Barone E, Mannucci PM (1978) Coexistence of haemophilia A and von Willebrand’s disease in the same kindred. Scand J Haematol 20: 423

    PubMed  CAS  Google Scholar 

  • Geiger MT, Rath CE (1963) Occurence of two hemorrhagic disorders with antihemophilic factor (AHF) deficiency in the same family: classical hemophilia and von Willenbrand’s disease. J Lab Clin Med 61: 424

    PubMed  CAS  Google Scholar 

  • George JN, Onofre AR (1982) Human platelet surface binding of endogenous secreted factor VIII-von Willebrand factor and platelet factor 4. Blood 59: 194–197

    PubMed  CAS  Google Scholar 

  • George JN, Nurden AT, Phillips DR (1984) Molecular defects in interactions of platelets with the vessel wall. N Engl J Med 311: 1084–1098

    PubMed  CAS  Google Scholar 

  • Giddings JC (1980) Hereditary coagulation disorders: laboratory techniques. In: Thomson JM (ed) Blood coagulation and haemostasis, a practical guide, 2nd edn. Churchill, Livingstone Eding-burgh, pp 117–15

    Google Scholar 

  • Giddings JC, Evans DJ, Bloom AL (1979) Quantitation of factor VIII related antigen ( FVIII-RAG) using a laser nephelometer. Thromb Res 15: 847

    Google Scholar 

  • Gilchrist GS, Hagedorn AB, Owen CA Jr, Bowie EJW (1980) Management of patients with von Willebrand’s disease undergoing surgical procedures. In: Mammen EF, Barnhart MI, Lusher JM, Walsh RT (eds) Treatment of bleeding disorders. PJD Publications limited Westbury/NY 11590, p 83

    Google Scholar 

  • Girma JP, Ardaillou N, Meyer D et al. (1979) Fluid phase immunoradiometric assay for the detection of qualitative abnormalities of factor VIII/von Willebrand factor in variants of von Willebrand’s disease. J Lab Clin Med 93: 926–939

    PubMed  CAS  Google Scholar 

  • Graham JB (1979) Genotype assignment (carrier detection) in the hemophilias. Clin Haematol 8: 115–14

    PubMed  CAS  Google Scholar 

  • Gralnick HR (1978) Factor VIII/von Willebrand factor protein galactose, a cryptic determinant of von Willebrand factor activity. J Clin Invest 62: 496–499

    PubMed  CAS  Google Scholar 

  • Gralnick HR, Coller BS, Sultan Y (1975) Studies of the human factor VIII/von Willebrand factor protein. III. Qualitative defects in von Willebrand’s disease. J Clin Invest 56: 814–827

    Google Scholar 

  • Gralnick HR, Coller BR, Sultan Y (1976) Carbohydrate deficiency of the factor VIII von Willebrand protein in von Willebrand’s disease variants. Science 192: 56–59

    PubMed  CAS  Google Scholar 

  • Gralnick HR, Coller BS, Shulman NR et al. (1977a) Factor VIII. Ann Intern Med 86: 598–616

    Google Scholar 

  • Gralnick HR, Sultan Y, Coller BS ( 1977 b) von Willebrand’s disease. Combined qualitative and quantitative abnormalities. N Engl J Med 296: 1024–1030

    Google Scholar 

  • Gralnick HR, Williams SB, Morisato DK (1981a) Effect of the multimeric structure of the factor VIII/von Willebrand factor protein on binding to platelets. Blood 58: 387–397

    PubMed  CAS  Google Scholar 

  • Gralnick HR, Williams SB, Shafer B, Corash L (1981b) von Willebrand’s disease (vWd) with normal ristocetin-induced platelet aggregation (RIPA); abnormal platelets and abnormal factor VIII/von Willebrand factor (FVIII/VWF) protein. Blood [Suppl 1] 58:193 a

    Google Scholar 

  • Gralnick HR, Cregger MC, Williams SB (1982 a) Characterization of the defect of the factor VIII/von Willebrand factor protein in von Willebrand’s disease. Blood 59: 542–548

    Google Scholar 

  • Gralnick HR, Williams SB, Shafer BC, Corash L (1982b) Factor VIII/von Willebrand factor binding to von Willebrand’s disease platelets. Blood 60: 328–332

    PubMed  CAS  Google Scholar 

  • Gralnick HR, Williams S, Rick M (1983) The role of Carbohydrate in the maintainance of the multimeric structure of the factor VIII/vWF protein. Thromb Haemostas 50: 318 (abstract)

    Google Scholar 

  • Green D (1979) Role of the von Willebrand factor in atherogenesis. Artery 5: 262–272

    PubMed  CAS  Google Scholar 

  • Green D, Potter EV (1976 a) Failure of AHF concentrate to control bleeding in von Willebrand’s disease. Am J Med 60: 357–360

    Google Scholar 

  • Green D, Potter EV (1976b) Platelet bound ristocetin aggregating factor in normal subjects and patients with von Willebrand’s disease. J Lab Clin Med 87: 976

    PubMed  CAS  Google Scholar 

  • Green D, Reynolds N (1977) Double-antibody radioimmunoassay for factor VHI-related antigen. Clin Chem 23: 1648–1653

    PubMed  CAS  Google Scholar 

  • Griggs TR, Cooper HA, Webster WF, Wagner RH, Brinkhous KM (1973) Plasma aggregating factor (bovine) for human platelets: a marker for study of antihemophilic and von Willebrand factors. Proc Natl Acad Sei USA 70: 2814

    CAS  Google Scholar 

  • Griggs TR, Webster WP, Cooper HA (1974) von Willebrand factor: Gene dosage relationships and transfusion response in bleeder swine-A new bioassay. Proc Natl Acad Sei USA 71: 2087–209

    Google Scholar 

  • Griggs TR, Potter J, McClanahan SB, Webster WB, Brinkhous KM (1977) Macromolecular factor VIII complex: Functional and structural heterogeneity observed in von Willebrand swine with transfusion. Proc Natl Acad Sei USA 74: 759–763

    Google Scholar 

  • Griggs TR, Reddick RL, Sultzer D, Brinkhous KM (1981) Susceptibility to atherosclerosis in aortas and coronary arteries of swine with von Willebrand’s disease. Am J Pathol 102: 137–145

    PubMed  CAS  Google Scholar 

  • Gross R, Mammen E (1958) Über Pseudohaemophilie, Angiohaemophilie, von Willebrand Jürgens-’sche Krankheit und verwandte hämorrhagische Diathesen. Klin Wochenschr 36: 112

    Google Scholar 

  • Hagedorn AB (1971) Von Willebrand’s disease. JAMA 216: 991

    PubMed  CAS  Google Scholar 

  • Hampton JW, Shinada S, Drummond M (1980) Von Willebrand’s disease: Perspectives in treatment. In: Mammen EF, Barnhart MI, Lusher JM, Walsh RT (eds) Treatment of bleeding disorders. PJD Publications Limited Westbury/NY 11590, p 71

    Google Scholar 

  • Handin RI, Moloney WC (1974) Antibody-induced von Willebrand’s disease. Blood 44: 933

    Google Scholar 

  • Handin RI, Martin V, Moloney WC (1976) Antibody-induced von Willebrand’s disease: A newly defined inhibitor syndrome. Blood 48: 393

    Google Scholar 

  • Hanna W, McCarroll D, McDonald T, Painter P, Tuller J, Chen J, Lange R (1981) Variant von Willebrand’s disease and pregnancy. Blood 58: 873

    PubMed  CAS  Google Scholar 

  • Harrison RL, McKee PA (1984) Estrogen stimulates von Willebrand factor production by cultured endothelial cells. Blood 63: 657–665

    PubMed  CAS  Google Scholar 

  • Hartmann W, Henning J (1982) Die Qualität von F-VIII-Konzentraten auf dem deutschen Arzneimittelmarkt. Die Ortskrankenkasse 64: 1–13

    Google Scholar 

  • Hawiger J, Fujimoto T, Ohara S (1981) The contrasting effect of thrombin and prostacyclin on availability of the receptor for factor VIIIVWF on human platelets. Clin Res 29: 571A

    Google Scholar 

  • Heimburger N, Schwinn H, Kumpe G, Herchenhan B (1977) F VIII-Konzentrate-Fortschritte in der Entwicklung. Pharmazeutische Zeitung 122: 1382

    CAS  Google Scholar 

  • Hellem AJ (1970) Platelet adhesiveness in von Willebrand’s disease. A study with a new modification of the glass bead filter method. Scand J Haematol 7: 374

    PubMed  CAS  Google Scholar 

  • Hill C, Taylor J J (1968) von Willebrand’s disease in obstetrics and gynaecology. J Obstet Gyn Brit Cwlth 75: 453

    Google Scholar 

  • Hill FGH, Enoyat MS, George AJ (1983) Investigation including VIIIR:Ag multimeric analysis of a large kindred with type IIA von Willebrand’s disease showing a dominant inheritance and similar gene expression in four generations. Thromb Haemostas 50: 735–739

    CAS  Google Scholar 

  • Holland L, Adamson A, Ingram GIC, Chalmers DG (1980) Acquired von Willebrand’s syndrome. Short communication. Br J Haematol 45: 161–164

    Google Scholar 

  • Holmberg L, Nilsson IM (1972) Genetic variants of von Willebrand’s disease. Br Med J 111: 317–32

    Google Scholar 

  • Holmberg L, Nilsson IM (1974) AHF related protein in clinical praxis. Scand J Haematol 10: 12–16

    Google Scholar 

  • Holmberg L, Nilsson IM (1979) VIIIR:Ag in platelets from patients with various forms of von Willebrand’s disease. Thromb Haemostas 42: 1033

    Google Scholar 

  • Holmberg L, Mannucci PM, Turesson I et al. (1974) Factor VIII antigen in the vessel walls in von Willebrand’s disease and hemophilia. Scand J Haematol 13: 33–38

    PubMed  CAS  Google Scholar 

  • Holmberg L, Borge L, Nilsson IM (1981) Factor VIII:C and VIIICAg response in a patient with haemophilia A and von Willebrand’s disease after administration of different factor VIII concentrates or plasma. Br J Haematol 47: 587

    PubMed  CAS  Google Scholar 

  • Holmberg L, Nilsson IM, Borge L, Gunnarsson M, Sjörin E (1983) Platelet aggretation induced by l-desamino-d-arginine Vasopressin ( DDAVP) in Type IIB von Willebrand’s disease. N Engl J Med 309: 816

    Google Scholar 

  • Howard MA, Firkin BG (1971) Ristocetin-A new tool in the investigation of platelet aggregation. Thromb Diath Haemorrh 26: 362–369

    PubMed  CAS  Google Scholar 

  • Howard MA, Sawers RJ, Firkin BG (1973) Ristocetin: A means of differentiating von Willebrand’s disease into two groups. Blood 41: 687–690

    Google Scholar 

  • Howard MA, Montgomery DC, Hardisty RM (1974) Factor VHI-related antigen in platelets. Thromb Res 4: 617–62

    PubMed  CAS  Google Scholar 

  • Howard MA, Hendrix L, Firkin BG (1979) Further studies on the factor VIII of a patient with a variant form of von Willebrand’s disease. Thromb Res 14: 609–619

    PubMed  CAS  Google Scholar 

  • Howard MA, Salem HH, Thomas KB et al. (1982) Variant von Willebrand’s disease type B-revisited. Blood 60: 1420–142

    PubMed  CAS  Google Scholar 

  • Hoyer LW (1972) Immunological studies of antihemophilia factor (AHF, factor VIII). IV. Radioimmunoassay of AHF antigen. J Lab Clin Med 80: 822–833

    PubMed  CAS  Google Scholar 

  • Hoyer LW (1981) The factor VIII complex: Structure and function. Blood 58: 1–13

    PubMed  CAS  Google Scholar 

  • Hoyer LW (1982) The Assessment of von Willebrand’s disease. In: Bloom AL (ed) The hemophilias. Methods in hematology, vol 5. Churchill Livingstone, Edinburgh London Melbourne New York, pp 106–12

    Google Scholar 

  • Hoyer LW, Shainoff JR (1980) Factor VHI-related protein circulates in normal human plasma as high molecular weight multimers. Blood 55: 1056–1059

    PubMed  CAS  Google Scholar 

  • Hoyer LW, Trabold NC (1981) The effect of thrombin on human factor VIII. Cleavage of the factor VIII procoagulant protein during activation. J Lab Clin Med 97: 50–64

    Google Scholar 

  • Hoyer LW, Santos RP de los, Hoyer JR (1973) Antihemophilic factor antigen: Localization in endothelial cells by immunofluorescent microscopy. J Clin Invest 52: 2737–2744

    Google Scholar 

  • Hoyer LW, Rizza CR, Tuddenham EGD, Carta CA, Armitage H, Rotblat F (1983) Von Willebrand factor multimer patterns in von Willebrand’s disease. Br J Haematol 55: 493

    PubMed  CAS  Google Scholar 

  • Ingram GIC (1978) Classification of von Willebrand’s disease. Lancet 11: 1364

    Google Scholar 

  • Ingram GIC, Kingston PJ, Leslie J et al. (1971) Four cases of acquired von Willebrand’s syndrome. Br J Haematol 21: 189

    PubMed  CAS  Google Scholar 

  • Ingram GIC, Prentice CRM, Forbes CD et al. (1973) Low factor VHI-like antigen in acquired von Willebrand’s syndrome and response to treatment. Br J Haematol 25: 137

    PubMed  CAS  Google Scholar 

  • Italian Working Group (1977) Spectrum of von Willebrand’s disease: A study of 100 cases. Br J Haematol 35: 101–11

    Google Scholar 

  • Ivy AC, Nelson D, Bucher G (1941) The standardization of certain factors in the cutaneous “venostasis” bleeding time technique. J Lab Clin Med 26: 1812–1822

    Google Scholar 

  • Jaffe EA, Hoyer LW, Nachman RL (1973) Synthesis of antihemophilic factor antigen by cultured human endothelial cells. J Clin Invest 52: 2757–2764

    PubMed  CAS  Google Scholar 

  • Jaffe EA, Hoyer LW, Nachman RL (1974) Synthesis of von Willebrand factor by cultured human endothelial cells. Proc Natl Acad Sei USA 71: 1906–1909

    CAS  Google Scholar 

  • Jakab T, Pflugshaupt R, Furlan M, Beck EA (1978) Variable degradation of factor VHI-related protein in lyophilised concentrates of antihaemophilic factor ( AHF ). Vox Sang 35: 36

    Google Scholar 

  • Jenkins CSP, Meyer D, Larrieu MJ (1976 a) Interaction of ristocetin and von Willebrand factor. Thromb Haemostas 35: 752–753

    Google Scholar 

  • Jenkins CSP, Phillips DR, Clemetson KJ (1976b) Platelet membrane glycoproteins implicated in ristocetin-induced aggregation. Studies of proteins on platelets from patients with Bernard-Soulier syndrome and von Willebrand’s disease. J Clin Invest 57: 112–124

    Google Scholar 

  • Johnson AJ, Karpatkin MH, Newman J (1971) Clinical investigation of intermediate and high-purity antihaemophilic factor (factor VIII) concentrates. Br J Haematol 21: 21

    PubMed  CAS  Google Scholar 

  • Joist HJ, Cowan JF, Zimmerman TS (1978) Acquired von Willebrand’s disease: Evidence for a quantitative and qualitative factor VIII disorder. N Engl J Med 298: 988–991

    Google Scholar 

  • Jürgens J (1969) Zur Klinik der von Willebrand Jürgens Syndrome. Hämat und Bluttransfusion 6: 164

    Google Scholar 

  • Jürgens R, Lehmann W, Wegelius D (1957) Mitteilung über Mängel an antihaemophilem Globulin ( Faktor VIII) bei der Aalandischen Thrombopathie (von Willebrand Jürgens ). Thromb Diath Haemorrh 1: 257–260

    Google Scholar 

  • Kalogjera V, Owen WG (1978) Identity of bovine platelet aggregating factor and ristocetin-Willebrand factor. Thromb Res 13: 857

    PubMed  CAS  Google Scholar 

  • Kao KJ, Pizzo SV, McKee P (1979 a) Demonstration and characterization of specific binding sites for factor VIII/von Willebrand factor on human platelets. J Clin Invest 63: 656–664

    Google Scholar 

  • Kao KJ, Pizzo SV, McKee P (1979b) Platelet receptors for human factor VIII/von Willebrand factor protein: Functional correlation of receptor occupancy and ristocetin-induced platelet aggregation. Proc Natl Acad Sei USA 76: 5317–5320

    Google Scholar 

  • Kasper CK, Hoag MS, Aggeier PM, Stone S (1964) Blood clotting factors in pregnancy: factor VIII concentrations in normal and AHF-deficient women. Obstet Gynecol 24: 242

    PubMed  CAS  Google Scholar 

  • Kass L, Ratnoff OD, Leon MA (1969) Studies on the purification of antihemophilic factor (factor VIII). I. Precipitation of antihemophilic factor by concanavalin A. J Clin Invest 48: 351–358

    Google Scholar 

  • Katzmann JA, Mujwid DK, Miller RS, Fass DN (1981) Monoclonal antibodies to von Willebrand’s factor: Reactivity with porcine and human antigens. Blood 58: 530–535

    Google Scholar 

  • Kelton JG, Bishop J, Carter CJ, Hirsh J (1980) A comparison of the quantitative ristocetin von Willebrand factor assay by using fresh and fixed platelets. Thromb Res 18: 477–483

    PubMed  CAS  Google Scholar 

  • Kernoff LM, Rose AG, Hughes J, Jacobs P (1981) Autopsy findings in an elderly man suffering from severe von Willebrand’s disease. Thromb Haemostas 46: 714

    CAS  Google Scholar 

  • Kernoff PBA, Gruson R, Rizza CR (1974) A variant of factor VHI-related antigen. Br J Haematol 26: 435–44

    PubMed  CAS  Google Scholar 

  • Kessler CM, Floyd CM, Rick ME et al. (1984) Collagen-factor VIII/von Willebrand factor protein interaction. Blood 63: 1291–1298

    PubMed  CAS  Google Scholar 

  • Kinoshita S, Harrison J, Lazerson J, Abildgaard Ch (1984) A new variant of dominant type II von Willebrand’s disease with aberrant multimeric pattern structure of factor VHI-related antigen, ( Type II D ). Blood 63: 1369–1371

    Google Scholar 

  • Köhler M, Hellstern P, Reiter B et al. (1984a) Behandlung des von Willebrand Jürgens Syndroms mit „hepatitissicheren“Faktor VIII-Konzentraten. Dtsch Med Wochenschr 109: 1800–1805

    PubMed  Google Scholar 

  • Köhler M, Hellstern P, Reiter B et al. (1984b) The subcutaneous administration of the Vasopressin analogue l-Desamino-D-Arginine Vasopressin in patients with von Willebrand’s disease and hemophilia. Klin Wochenschr 62: 543–548

    PubMed  Google Scholar 

  • Korninger Ch, Niessner H (1979) Systemische Therapie des von Willebrand Jürgens Syndroms mit Plasmaderivaten. In: Landbeck G, Marx R, Stolte HP (Hrsg) 10. Haemophilie-Symposion, Hamburg 1979. Pharmazeutische Verlagsgesellschaft, S 93–108

    Google Scholar 

  • Korninger Ch, Niessner H, Thaler E, Lechner K (1980) Wirkung von DDAVP auf Fibrinolyse-und Gerinnungsparameter. Untersuchung an Normalpersonen, Haemophilen und Patienten mit von Willebrand Jürgens Syndrom. In: Sutor AH (ed) DDAVP in bleeding disorders (1st Int Symposium on DDAVP in Bleeding Disorders, Münster 1979 ). Schattauer, Stuttgart New York, pp 55–6

    Google Scholar 

  • Korninger Ch, Niessner H, Lechner K (1981) Impaired fibrinolytic response to DDAVP and venous occlusion in a sub-group of patients with von Willebrand’s disease. Thromb Res 23: 365–374

    PubMed  CAS  Google Scholar 

  • Koutts J, Lavergne JM, Meyer D (1977) Immunological evidence that human factor VIII is composed of two linked moieties. Br J Haematol 37: 415–428

    PubMed  CAS  Google Scholar 

  • Koutts J, Walsh PN, Plow EF et al. (1978) Active release of human platelet factor VHI-related antigen by adenosine diphosphate, collagen and thrombin. J Clin Invest 62: 1255–1263

    PubMed  CAS  Google Scholar 

  • Koutts J, Howard MA, Firkin BG (1979) Factor VIII physiology and pathology in man. In: Brown EB (ed) Progress in hematology. Grune and Stratton, New York, pp 115–146

    Google Scholar 

  • Krishnamurthy M, Miotti AB (1977) Von Willebrand’s disease and pregnancy. Obstet Gynecol 49: 244

    PubMed  CAS  Google Scholar 

  • Krizek DM, Rick ME, Williams SB, Gralnick HR (1983) Cryoprecipitate transfusion in variant von Willebrand’s disease and thrombocytopenia. Ann Intern Med 98: 484

    PubMed  CAS  Google Scholar 

  • Kröniger A, Kumpe A, Wormsbächer W, Herchenhahn B (1982) Faktor-VIII-Konzentrate. Med Welt 33: 1027

    PubMed  Google Scholar 

  • Kudo Y, Yokoyama M, Kimura A, Takamatsu H (1977) A case of combined deficiency of factor IX and factor VIII with prolonged bleeding time. Jap J Clin Hematol 18: 81

    CAS  Google Scholar 

  • Larrieu MJ, Soulier JP (1953) Deficit en facteur antihemophilique A chez une fille associe a un trouble du saignement. Rev Hematol 8: 361–37

    PubMed  CAS  Google Scholar 

  • Larrieu MJ, Caen JP, Meyer D et al. (1968) Congenital bleeding disorders with long bleeding time and normal platelet count. II. von Willebrand’s disease (report of 37 patients). Am J Med 45: 354 — 372

    PubMed  CAS  Google Scholar 

  • Laurell CB (1965) Antigen-antibody crossed electrophoresis. Anal Biochem 10: 358

    PubMed  CAS  Google Scholar 

  • Laurell CB (1966) Quantitative estimation of protein by electrophoresis in agarose gel containing antibodies. Anal Biochem 15: 45

    PubMed  CAS  Google Scholar 

  • Lazarchick J, Hoyer LW (1978) Immunoradiometric measurement of the factor VIII procoagulant antigen. J Clin Invest 62: 1048–1052

    PubMed  CAS  Google Scholar 

  • Lechner K (1982) Blutgerinnungsstörungen-Laboratoriumsdiagnose hämatologischer Erkrankungen. Springer, Berlin Heidelberg New York

    Google Scholar 

  • Lechner K, Niessner H, Stych H (1975) Zur Vermeidung postoperativer Blutungszwischenfälle bei Operationen im Hals-, Nasen-, Ohrenbereich. Österr Ärztezeitung 30: 1310

    Google Scholar 

  • Legaz ME, Schmer G, Counts RB, Davie EW (1973) Isolation and characterization of human factor VIII (antihemophilic factor). J Biol Chem 248: 3946–3955

    PubMed  CAS  Google Scholar 

  • Legrand YJ, Rodriguez-Zeballos A, Kartalis G et al. (1978) Adsorption of factor VIII antigen-activity complex by collagen. Thromb Res 13: 909

    PubMed  CAS  Google Scholar 

  • Lehmann W (1959) Neuere Untersuchungen zur Thrombopathie (von Willebrand-Jürgens) auf den Älands-Inseln (Finnland). Acta Genet Med [Suppl] 2: 68

    Google Scholar 

  • Leone G, Pola P, Guerra G et al. (1974) Sindrome di von Willebrand acquisita in corso di malattia disreattiva. Haematologica 59: 212

    PubMed  CAS  Google Scholar 

  • Leone G, Moneta E, Paparotti G, Boni P (1975) von Willebrand’s disease in pregnancy. N Engl J Med 293: 456

    Google Scholar 

  • Leupin L, Beck EA, Furlan M, Bucher U (1983) Hämostasestörung mit verminderter Aktivität des von Willebrand-Faktors bei myeloproliferativen Syndromen. Schweiz Med Wochenschr 113: 713–71

    PubMed  CAS  Google Scholar 

  • Lian ECY, Deykin D (1976) Diagnosis of von Willebrand’s disease. A comparative study of diagnostic tests on nine families with von Willebrand’s disease and its differential diagnosis from hemophilia and thrombocytopathy. Am J Med 60: 344–356

    Google Scholar 

  • Lopez Fernandez MF, Ginsberg MH, Ruggeri ZM (1982) Multimeric structure of platelet factor VIII/von Willebrand factor: The presence of larger multimers and their reassociation with thrombin stimulated platelets. Blood 60: 1132–1138

    Google Scholar 

  • Ludlam CA, Peake IR, Allen N et al. (1980) Faktor VIII and fibrinolytic response to deamino–d-arginine vasopressin in normal subjects and dissociate response in some patients with haemophilia and von Willebrand’s disease. Br J Haematol 45: 499–511

    PubMed  CAS  Google Scholar 

  • Lynch DC, Williams R, Kirby E et al. (1982) Mechanism of factor VIIIR (von Willebrand factor) synthesis by bovine endothelial cells. Clin Res 30: 506a

    Google Scholar 

  • Lynch DC, Williams R, Zimmerman TS, Kirby E, Livingston DM (1983) Biosynthesis of the subunits of factor VIIIR by bovine aortic endothelial cells. Proc Natl Acad Sei USA 80: 2738

    CAS  Google Scholar 

  • MacFarlane DE, Stibbe J, Kirby EP et al. (1975) A method for assaying von Willebrand factor (ristocetin cofactor). Thromb Diath Haemorrh 34: 306–308

    PubMed  CAS  Google Scholar 

  • Mancini G, Carbonara AO, Heremans JF (1965) Immunochemical quantitation of antigens by simple radial immunodiffusion. Immunochemistry 2: 235

    PubMed  CAS  Google Scholar 

  • Mannucci PM, Ruggeri ZM, Gagantelli G (1971) Nervous regulation of factor VIII levels in man. Br J Haematol 20: 195

    PubMed  CAS  Google Scholar 

  • Mannucci PM, Pareti FI, Ruggeri ZM (1974) Enhanced factor VIII activity in von Willebrand’s disease. N Engl J Med 290: 1259

    PubMed  CAS  Google Scholar 

  • Mannucci PM, Äberg M, Nilsson IM, Robertson B (1975) Mechanism of plasminogen activator and factor VIII increase after vasoactive drugs. Br J Haematol 30: 81–93

    PubMed  CAS  Google Scholar 

  • Mannucci PM, Meyer D, Ruggeri ZM, Koutts J, Ciavarella N, Lavergne JM (1976 a) Precipitating antibodies in von Willebrand’s disease. Nature 262: 141–142

    Google Scholar 

  • Mannucci PM, Pareti FI, Holmberg L et al. (1976b) Studies on the prolonged bleeding time in von Willebrand’s disease. J Lab Clin Med 88: 662–671

    PubMed  CAS  Google Scholar 

  • Mannucci PM, Ruggeri ZM, Pareti FI, Capitanio A (1977a) l-deamino–8-d-arginine vasopressin: A new pharmacological approach to the management of haemophilia and von Willebrand’s disease. Lancet 1: 869–872

    Google Scholar 

  • Mannucci PM, Ruggeri ZM, Pareti FI, Capitanio A (1977 b) DD A VP in haemophilia. Lancet II: 1171 (letter)

    Google Scholar 

  • Mannucci PM, Canciani MT, Rota L, Donovan BS (1981 a) Response of factor VIII/von Willebrand factor to DDAVP in healthy subjects and patients with haemophilia A and von Willebrand’s disease. Br J Haematol 47: 283–293

    Google Scholar 

  • Mannucci PM, Ruggeri ZM, Ciavarella N (1981b) Precipitating antibodies to factor VIII/von Willebrand factor in von Willebrand’s disease: Effects on replacement therapy. Blood 57: 25–31

    Google Scholar 

  • Mannucci PM, Lombardi R, Pareti FI, Solinas S, Mazzucconi MG, Mariani G (1983) A variant of von Willebrand’s disease (vWd) characterized by recessive inheritance and missing triplet structure of von Willebrand factor multimers. Blood 62: 1000–1005

    PubMed  CAS  Google Scholar 

  • Mannucci PM, Bloom AL, Larrieu MJ, Nilsson IM (1984) On behalf of the European thrombosis research organization (ETRO). Atherosclerosis and von Willebrand factor. 1. Prevalence of severe von Willebrand’s disease in Western Europe and Israel. Br J Hemat 57: 163–169

    Google Scholar 

  • Mant MJ, Hirsh J, Gauldie J et al. (1973) Von Willebrand’s syndrome presenting as an acquired bleeding disorder in association with a monoclonal gammopathy. Blood 42: 429–436

    PubMed  CAS  Google Scholar 

  • Maragall S, Ordinas A, Rodriguez M, Liendo F, Castillo R (1979) Inhibition to Willebrand factor in von Willebrand disease. Thromb Res 14: 495–501

    PubMed  CAS  Google Scholar 

  • Marco L de, Shapiro SS (1981) Properties of human asialofactor VIII. A ristocetin-independent platelet-aggregating agent. J Clin Invest 68: 321–328

    Google Scholar 

  • Markwardt F (1983) Gefäßwand und Fibrinolyse. Arzneimittelforsch 33: 1370

    CAS  Google Scholar 

  • Marx R (1959) The problem of differentiating pseudohemophilias. In: Brinkhous KM, Nicola P de (eds) Hemophilia and other hemorrhagic states. University of North Carolina Press, Chapel Hill, pp 108–12

    Google Scholar 

  • Marx R, Jean G (1964) Zur Pathogenese der von Willebrand Jürgens Syndrome. Eine klinische und submikroskopische Studie. Klin Wochenschr 42: 491

    Google Scholar 

  • McCarroll DR, Ruggeri ZM, Montgomery RR (1984) The effect of DDAVP on plasma levels of von Willebrand antigen II in normal individuals and patients. Blood 63: 532–535

    PubMed  CAS  Google Scholar 

  • McMichael AJ, Rust NA, Pilch JR, Sochynsky R, Morton J, Mason DY, Ruan C, Tobelem G, Caen J (1981) Monoclonal antibody to human platelet glycoprotein I. I. Immunological studies. Br J Haematol 49: 501

    Google Scholar 

  • Meili EQ, Straub PW, Frick PG (1969) Zur Pathogenese und Behandlung der von Willebrand’schen Krankheit. Schweiz Med Wochenschr 99: 1805

    PubMed  CAS  Google Scholar 

  • Meyer D, Baumgartner HR (1983) Role of von Willebrand factor in platelet adhesion to the subendothelium. Br J Haematol 54: 1–9

    PubMed  CAS  Google Scholar 

  • Meyer D, Lavergne JM, Larrieu MJ, Josso F (1972) Crossreacting material in congenital factor VIII deficiences ( Hemophilia A and von Willebrand’s disease ). Thromb Res 1: 183–195

    Google Scholar 

  • Meyer D, Jenkins CSP, Dreyfus MD (1974) Willebrand factor and ristocetin. II. Relationship between Willebrand factor, Willebrand antigen and factor VIII activity. Br J Haematol 28: 579

    Google Scholar 

  • Meyer D, Frommel D, Larrieu MJ, Zimmerman TS (1979) Selective absence of large forms of factor VIII/von Willebrand factor in acquired von Willebrand’s syndrome. Response to transfu-sion. Blood 54: 600–60

    Google Scholar 

  • Meyer D, Obert B, Pietu G et al. (1980) Multimeric structure of factor VIII/von Willebrand factor in von Willebrand’s disease. J Lab Clin Med 95: 590–602

    PubMed  CAS  Google Scholar 

  • Meyer D, Baumgartner HR, Edgington TS (1981) Effect of hybridoma antibodies to human factor VIII/von Willebrand factor on the adhesion of platelets to the subendothelium. Blood 58: 237a (abstract)

    Google Scholar 

  • Meyer D, Lavergne JM, Baumgartner HR, Tobelem G, Pietu G, Edgington TS (1983) Monoclonal antibodies to human von Willebrand factor: Role of intramolecular loci in mediation of platelet adhesion to the subendothelium. Thromb Haemostas 50: 191 (abstract)

    Google Scholar 

  • Mielke CH, Kaneshiro MM, Mäher IA et al. (1969) The standardized normal Ivy bleeding time and its prolongation by aspirin. Blood 34: 204–215

    PubMed  Google Scholar 

  • Miller CH, Graham JG, Goldin LR, Elston RC ( 1979 a) Genetics of classic von Willebrand’s disease. I. Phenotypic variation within families. Blood 54: 117

    Google Scholar 

  • Miller CH, Graham JB, Goldin LR, Elston RC ( 1979 b) Genetics of classic von Willebrand’s disease. II. Optimal assignment of the heterozygous genotype (diagnosis) by discriminant analysis. Blood 54: 137

    Google Scholar 

  • Miller JL, Castella A (1982) Platelet-type von Willebrand’s disease: Characterization of a new bleeding disorder. Blood 60: 790–794

    Google Scholar 

  • Miller JL, Kupinsi JM, Castella A, Ruggeri ZM (1983) Von Willebrand factor binds to platelets and induces aggregation in platelet-type but not type IIB von Willebrand disease. J Clin Invest 72: 1532–154

    PubMed  CAS  Google Scholar 

  • Minot GR (1928) A familial hemorrhagic condition associated with prolongation of the bleeding time. Am J Med Sei 175: 301

    Google Scholar 

  • Miyashita C, Hellstern P, Köhler M et al. (1984) In vitro characterization of commercial factor VIII concentrates longterm follow-up. Blut 48: 1–7

    Google Scholar 

  • Moake JL, Olson JD, Troll JH, Tang SS, Funicella T, Peterson DM (1980) Binding of radioiodinated human von Willebrand factor to Bernard-Soulier Syndrom, thrombasthenic and von Willebrand platelets. Thromb Res 19: 21

    PubMed  CAS  Google Scholar 

  • Moake JL, Rudy CK, Troll JH, Weinstein MJ, Colannino NM, Azocar J, Seder RH, Hong SL, Deykin DL (1982) Unusually large plasma factor VIII:von Willebrand factor multimers in chronic relapsing thrombotic thrombocytopenic purpura. N Engl J Med 307: 1432–1435

    PubMed  CAS  Google Scholar 

  • Montgomery RR, Zimmerman TS (1978) von Willbrand’s disease antigen II (vW:Ag II). A new plasma and platelet antigen deficient in severe von Willebrand’s disease. J Clin Invest 61: 1498–150

    Google Scholar 

  • Montgomery RR, Zimmerman TS (1978) von Willbrand’s disease antigen II (vW:Ag II). A new plasma and platelet antigen deficient in severe von Willebrand’s disease. J Clin Invest 61: 1498–150

    Google Scholar 

  • Morisato DK, Gralnick HR (1980) Selective binding of the factor VIII/von Willebrand factor protein to human platelets. Blood 55: 9–15

    PubMed  CAS  Google Scholar 

  • Mourik JA van, Bouma BN, Bruyere WT la et al. (1974) Factor VIII, a series of homologous oligomers and a complex of two proteins. Thromb Res 4: 155–164

    PubMed  Google Scholar 

  • Müntz RH, Ekert M, Helliger H (1974) Properties of postinfusion factor VIII in von Willebrand’s disease. Thromb Res 5: 111

    PubMed  Google Scholar 

  • Nachman RL, Jaffe EA (1975) Subcellular platelet factor VIII antigen and von Willebrand factor. J Exp Med 141: 1101–1113

    PubMed  CAS  Google Scholar 

  • Nachman R, Levine R, Jaffe EA (1977) Synthesis of factor VHI-antigen by cultured guineapig megakaryocytes. J Clin Invest 60: 914–921

    PubMed  CAS  Google Scholar 

  • Nielsen EG, Svejgard A (1967) von Willebrand’s disease associated with intermittent thrombocytopenia. Lancet II: 966

    Google Scholar 

  • Niessner H (1972) Messung der Plättchenadhäsivität mit einer modifizierten Form der Hellem Ii-Methodik unter besonderer Berücksichtigung des von Willebrand-Jürgens-Syndrom. Thromb Diath Hämorrh 27: 434

    CAS  Google Scholar 

  • Niessner H (1976) Die Laboratoriumsdiagnostik des von Willebrand-Jürgens-Syndroms. Wien Klin Wochenschr 88: 221–23

    PubMed  CAS  Google Scholar 

  • Niessner H (1977) Two cases of acquired von Willebrand’s syndrome with associated monoclonal gammopathy. In: Workshop on Inhibitors of Factors VIII and IX, Jan 26th and 27th, 1976, Facultas, Wien, p 185

    Google Scholar 

  • Niessner H (1978) Das analytische Spektrum zur Diagnostik des von Willebrand Jürgens Syndroms. In: Heene DL (Hrsg) Immunologische Probleme der Blutgerinnung, von Willebrand Jürgens Syndrom. Verhandlungsbericht der Dtsch Arbeitsgemeinschaft für Blutgerinnungsforschung Giessen 1976. Schattauer, Stuttgart New York, S 199

    Google Scholar 

  • Niessner H (1981) Das erworbene von Willebrand Jürgens Syndrom. In: Scharrer J, Breddin K (Hrsg) Haemostase und Atherosklerose bei Haemophilie und von Willebrand-Syndrom. ( 14. Angiologisches Symposium in Kitzbühel.) Schattauer, Stuttgart, New York, S 39

    Google Scholar 

  • Niessner H (1982) Neue Erkenntnisse beim von Willebrand Jürgens Syndrom. Acta Med Austriaca [Suppl] 2: 44 — 50

    Google Scholar 

  • Niessner H (1983) Die Rolle von Faktor VIII bei der Interaktion von Plättchen und Gefäßendothel. Arzneimittelforsch 33: 1379–1383

    Google Scholar 

  • Niessner H, Brenner B (1982) Zur Methodik der Bestimmung des Ristocetin-Cofaktors. In: Landbeck G, Marx R, Stolte HP (Hrsg), 11. Haemophilie Symp Hamburg 1980. Pharmazeutische Verlags-Gesellschaft, München, S 91–105

    Google Scholar 

  • Niessner H, Korninger CH (1983) DD A VP-an alternative in the management of mild hemophilia A and von Willebrand’s disease. Wien Klin Wochenschr 95: 753–757

    PubMed  CAS  Google Scholar 

  • Niessner H, Lechner K (1979) Failure of cryoprecipitate to normalize platelet retention in heriditary and acquired von Willebrand’ disease. Thromb Haemostas 42: 442 (abstract)

    Google Scholar 

  • Niessner H, Korninger CH, Lechner K (1981) Impaired fibrinolytic response to DDAVP and venous occlusion in a subgroup of patients with von Willebrand’s disease. Thromb Haemostas 46: 123 (abstract)

    Google Scholar 

  • Nieuwenhuis HK, Sixma JJ (1983) Bleeding time measurements. In: Harker LA, Zimmerman ThS (eds) Measurements of platelet function, methods in hematology, vol 8. Churchill Livingstone, Edinburgh London Melbourne New York, pp 26–45

    Google Scholar 

  • Nilsson IM (1977) Von Willebrand’s disease-fifty years old. Acta Med Scand 201: 497

    PubMed  CAS  Google Scholar 

  • Nilsson IM (1978) Report of working party on factor VHI-related antigens. Thromb Haemostas 39: 511–52

    CAS  Google Scholar 

  • Nilsson IM, Blomback M (1963) Von Willebrand’s disease in Sweden. Occurence, pathogenesis and treatment. Thromb Diath Haemorrh [Suppl 2] 9: 103

    CAS  Google Scholar 

  • Nilsson IM, Hedner U (1977) Characteristics of various factor VIII concentrates used in treatment of haemophilia A. Br J Haematol 37: 543–557

    PubMed  CAS  Google Scholar 

  • Nilsson IM, Holmberg L (1979) Von Willebrand’s disease today. Clin Haematol 8: 147

    PubMed  CAS  Google Scholar 

  • Nilsson IM, Robertson B (1968) Effect of venous occlusion on coagulation and fibrinolytic components in normal subjects. Thromb Diath Haemorrh 20: 397–408

    PubMed  CAS  Google Scholar 

  • Nilsson IM, Blomback M, Francken I von ( 1957 a) On an inherited autosomal hemorrhagic’ diathesis with antihemophilic globulin ( AHG) deficiency and prolonged bleeding time. Acta Med Scand 159: 35–5

    Google Scholar 

  • Nilsson IM, Blomback M, Jorpes E et al. (1957b) Von Willebrand’s disease and its correction with human fraction I-O. Acta Med Scand 159: 179–188

    PubMed  CAS  Google Scholar 

  • Nilsson IM, Blomback M, Blomback B (1959) Von Willebrand’s disease in Sweden. Its pathogenesis and treatment. Acta Med Scand 164: 263–278

    Google Scholar 

  • Nilsson IM, Magnusson S, Borchgrevink KC (1963) The Duke and Ivy methods for determination of the bleeding time. Thromb Diath Haemorrh 10: 223–234

    PubMed  CAS  Google Scholar 

  • Nilsson IM, Bergentz SE, Larsson SA (1979 a) Surgery in von Willebrand’ disease. Ann Surg 190: 746

    Google Scholar 

  • Nilsson IM, Walter H, Mikaelson M, Vilhardt H (1979b) Factor VIII concentrate prepared form DDAVP stimulated blood donor plasma. Scand J Haematol 22: 42

    PubMed  CAS  Google Scholar 

  • Nilsson IM, Holmberg L, Aberg M, Vilhardt H (1980a) The release of plasminogen activator and factor VIII after injection of DD AVP in healthy volunteers and in patients with von Willebrand’s disease. Scand J Haematol 24: 351

    CAS  Google Scholar 

  • Nilsson IM, Peake IR, Bloom AL, Meyer D, Veitkamp JJ, Green D (1980b) Report of the working party on factor VIII related antigen. Addendum: The relationship between ristocetin cofactor activity (VIII: RiCof) and factor VIII related antigen ( VIIIR: Ag) Thromb Haemostas 43: 167–168

    Google Scholar 

  • Nilsson IM, Vilhardt H, Holmberg L, Ästedt B (1982) Association between factor VIII related antigen and plasminogen activator. Acta Med Scand 211: 105–112

    PubMed  CAS  Google Scholar 

  • Noller KL, Bowie EJW, Kempers RD (1973) von Willebrand’s disease in pregnancy. Obstet Gynecol 41: 865

    Google Scholar 

  • Nyman D (1977) Interaction of collagen with the factor VIII antigenactivity von Willebrand factor complex. Thromb Res 11: 433

    PubMed  CAS  Google Scholar 

  • Nyman D (1980) von Willebrand factor dependent platelet aggregation and adsorption of factor VHI-related antigen by collagen. Thromb Res 17:209

    Google Scholar 

  • Nyman D, Eriksson AW, Blombäck M, Fronts RR, Wahlberg P (1981) Recent investigations of the first bleeder family in Aland (Finland) described by von Willebrand. Thromb Haemostas 45 /1: 73–7

    CAS  Google Scholar 

  • Ockelford PA, Chandrasekhara Menon N, Berry EW (1980) Clinical experience with arginine vasopressin ( DDAVP) in von Willebrand’s disease and mild haemophilia. New Zealand Med J 92: 375–37

    Google Scholar 

  • Ogata K, Saito H, Ratnoff OD (1983) The relationship of the properties of antihemophilic factor (factor VIII) that support ristocetin-induced platelet agglutination (factor VIIIR: RC) and platelet retention by glass beads as demonstrated by a monoclonal antibody. Blood 61: 27–35

    Google Scholar 

  • Olson JD, Brockway WJ, Fass DN et al. (1977) Purification of porcine and human ristocetin Willebrand factor. J Lab Clin Med 89: 1278–1294

    PubMed  CAS  Google Scholar 

  • Over J, Vlooswijk HAA, Sixma JJ (1977) Assay of F VHI-related antigen in a variant of von Willebrand’s disease. Thromb Haemostas 37: 367–370

    CAS  Google Scholar 

  • Over J, Bouma BN, Mourik JA van (1978) Heterogeneity of human factor VIII. I. Characterization of factor VIII present in the supernatant of cryoprecipitate. J Lab Clin Med 91: 32–46

    Google Scholar 

  • Over J, Sixma JJ, Bouma BN et al. (1981) Survival of iodine–12-labeled factor VIII in patients with von Willebrand’s disease. J Lab Clin Med 97: 332–344

    PubMed  CAS  Google Scholar 

  • Owen CA Jr, Bowie EJW, Zollman PE (1974) Carriers of porcine von Willebrand’s disease. Am J Vet Res 35: 245–24

    PubMed  Google Scholar 

  • Owen CA, Bowie EJW, Fass DN, Perez RA, Cole TL, Stewart M (1979) Hypofibrinogenemia-dysfibrinogenemia and von Willebrand’s disease in the same family. Mayo Clin Proc 54: 375

    PubMed  Google Scholar 

  • Owen WG, Wagner RH (1972) Antihemophilic factor: Separation of an active fragment following dissociation by salts or detergents. Thromb Diath Haemorrh 27: 502–515

    Google Scholar 

  • Paulssen MMP, Graaf-Wildschut M van de, Kolhorn A, Planje MC (1975) Radioimmunoassay of antihaemophilic factor (factor VIII) antigen. Clin Chim Acta 63: 349–353

    PubMed  CAS  Google Scholar 

  • Peake IR (1982) Immunoradiometric assays of factor VIII. In: Bloom AL (ed) The Hemophilias, Methods in Hematology 5: 92–105

    Google Scholar 

  • Peake IR, Bloom AL ( 1977 a) Abnormal factor VIII related antigen (FVIIIRAG) in von Willebrand’s disease (vWd): Decreased precipitation by concanavalin A. Thromb Haemostas 37: 361–362

    Google Scholar 

  • Peake IR, Bloom AL (1977 b) The use of an immunoradiometric assay for factor VHI-related antigen in the study of atypical von Willebrand’s disease. Thrombos Res 10: 27–32

    Google Scholar 

  • Peake IR, Bloom AL (1978) Immunoradiometric assay of procoagulant factor VHI-antigen in plasma and serum and its reduction in hemophilia-Preliminary studies on adult and fetal blood. Lancet 1: 473–47

    PubMed  CAS  Google Scholar 

  • Peake IR, Bloom AL, Giddings JC (1974) Inherited variants of factor VHI-related protein in von Willebrand’s disease. N Engl J Med 291: 113–117

    PubMed  CAS  Google Scholar 

  • Perkins HA (1967) Correction of the hemostatic defect in von Willebrand’s disease. Blood 30: 375–380

    PubMed  CAS  Google Scholar 

  • Perret BA, Furlan M, Beck EA (1979) Studies in factor VHI-related protein. II. Estimation of molecular size differences between factor VIII oligomers. Biochim Biophys Acta 578: 164–174

    Google Scholar 

  • Pickering NJ, Brody JI, Barrett MJ (1981) Von Willebrand syndromes and mitral-valve prolapse. N Engl J Med 305: 131

    PubMed  CAS  Google Scholar 

  • Piovella F, Nalli G, Malamani GD et al. (1978) Ultrastructural localization of factor VHI-antigen in human platelets, megakaryocytes and endothelial cells utilizing a ferritin-labelled antibody. Br J Haematol 39: 209–213

    PubMed  CAS  Google Scholar 

  • Pool JG, Shannon AE (1965) Production of high-potency concentrates of antihemophilic globulin in a closedbag system: Assay in vitro and in vivo. N Engl J Med 273: 1443

    Google Scholar 

  • Pool-Wilson PA (1972) Acquired von Willebrand’s syndrome and systemic lupus erythematosus. ProcRSoc Med 65: 561

    Google Scholar 

  • Potter EV, Chediak J, Green D (1976) Absence of ristocetin aggregation factor from the skin of a patient with von Willebrand’s disease. Lancet 1: 514

    PubMed  CAS  Google Scholar 

  • Praga C, Valentini L, Maiorano M, Cortellaro M (1974) A new automatic device for the standardized Ivy bleeding time. In: Mannucci PM (ed) Platelet function and thrombosis: A review of methods. Plenum Press, New York

    Google Scholar 

  • Prowse CV, Sas G, Gader AM A, Cort JH, Cash JD (1979) Specifity in the factor VIII response to vasopressin infusion in man. Br J Haematol 41: 437–447

    PubMed  CAS  Google Scholar 

  • Quick A J (1967) Teleangiectasia: its relationship to the Minot-von Willebrand syndrome. Am J Med Sci 254: 585

    PubMed  CAS  Google Scholar 

  • Quick AJ, Adlam RT (1963) Coexistence of von Willebrand’s disease and hemophilia in a family. JAMA 185: 635

    PubMed  CAS  Google Scholar 

  • Quick AJ, Hussey CV (1953) Hemophilic condition in the female. J Lab Clin Med 42: 929

    Google Scholar 

  • Ramsay DM, Macleod DAD, Buist TAS, Heading RC (1976) Persistent gastrointestinal bleeding due to angiodysplasia of the gut in von Willebrand’s disease. Lancet II: 275

    Google Scholar 

  • Ramsey R, Evatt BL (1979) Rapid assay for von Willebrand factor activity using formalin-fixed platelets and microtitration technic. Am J Clin Pathol 72: 996

    PubMed  CAS  Google Scholar 

  • Rand JH, Gordon RE, Sussman II (1980 a) Electron microscopic localization of factor VIII-related antigen in human blood vessels. Circulation 62: 111–169

    Google Scholar 

  • Rand HJ, Sussman II, Gordon RE, Chu SV, Solomon V (1980 b) Localization of factor-VHI-related antigen in human vascular subendothelium. Blood 55: 752

    Google Scholar 

  • Ratnoff OD (1978) Antihemophilic factor (factor VIII). Ann Intern Med 88: 403–409

    PubMed  CAS  Google Scholar 

  • Ratnoff OD, Bennett B (1973) Clues to pathogenesis of bleeding in von Willebrand’s disease. N Engl J Med 289: 1182–1183

    PubMed  CAS  Google Scholar 

  • Ratnoff OD, Saito H (1974) Bleeding in von Willebrand’s disease. N Engl J Med 290: 1089

    PubMed  CAS  Google Scholar 

  • Ratnoff OD, Slover CC, Poon MC (1976) Immunologic evidence that the properties of human antihemophilic factor (factor VIII) are attributes of a single molecular species. Blood 47: 657–667

    PubMed  CAS  Google Scholar 

  • Read MS, Shermer RW, Brinkhous KM (1978) Venom coagglutinin: An activator of platelet aggregation dependent on von Willebrand factor. Proc Natl Acad Sci USA 75: 4514–451

    Google Scholar 

  • Reddick RL, Griggs TR, Lamb MA, Brinkhous KM (1982) Platelet adhesion to damaged arteries. Comparison in normal and von Willebrand disease swine. Proc Natl Acad Sci USA 79: 5076

    Google Scholar 

  • Reisner HM, Katz HJ, Goldin LR et al. (1978) Use of a simple visual assay of Willebrand factor for diagnosis and carrier identification. Br J Haematol 40: 339–350

    PubMed  CAS  Google Scholar 

  • Reisner HM, Barrow ES, Graham JB (1979) Radioimmunoassay for coagulant factor VHI-related antigen ( VIIIC: Ag). Thromb Res 14: 235–239

    Google Scholar 

  • Report of a WHO Scientific group (1972) Inherited blood clotting disorders. WHO technical report 504. Geneva, WHO

    Google Scholar 

  • Rivard GE, Daviault MB (1978) A simplified assay for von Willebrand factor. Thromb Res 12: 677

    PubMed  CAS  Google Scholar 

  • Rivard GE, Daviault MB, Brault N, D’Aragon L, Raymond R (1977) von Willebrand’s disease associated with thrombocytopenia and a fast migrating factor VIII related antigen. Thromb Res 11: 507

    Google Scholar 

  • Rizza CR (1961) Effect of exercise on the level of antihemophilic factor in the human blood. J Physiol 156: 128

    PubMed  CAS  Google Scholar 

  • Rosborough TK, Swaim WR (1978) Acquired von Willebrand’s disease, platelet-release defect and angiodysplasia. Am J Med 65: 96–100

    PubMed  CAS  Google Scholar 

  • Ross R, Glomset JA (1976) The pathogenesis of atherosclerosis. N Engl J Med 295: 369–377

    PubMed  CAS  Google Scholar 

  • Roussi JH, Houbouyan LL, Alterescu R, Franc B, Goguel AF (1980) Acquired von Willebrand’s syndrome associated with hairy cell leukaemia. Br J Haematol 46: 503–506

    PubMed  CAS  Google Scholar 

  • Ruan C, Tobelem G, McMichael AJ, Drouet L, Legrand Y, Degos L, Kieffer N, Lee H, Caen JP (1981) Monoclonal antibody to human platelet glycoprotein I. II. Effects on human platelet function. Br J Haematol 49: 511

    Google Scholar 

  • Rubin H, Alter AA, Walker MR, Stefanyshyn M, Scott L, Miotti AB (1976) Evidence that the platelet retention factor is separate from the factor VHI-related antigen and factor VIII. J Lab Clin Med 88: 14

    PubMed  CAS  Google Scholar 

  • Ruggeri ZM, Zimmerman TS (1980) Variant von Willebrand’s disease: Characterization of two subtypes by analysis of multimeric composition of factor Vlll/von Willebrand factor in plasma and platelets. J Clin Invest 65: 1318–1325

    Google Scholar 

  • Ruggeri ZM, Zimmerman TS (1981) The complex multimeric composition of factor Vlll/von Willebrand factor. Blood 57: 1140–1143

    PubMed  CAS  Google Scholar 

  • Ruggeri ZM, Pareti FI, Bintadis P, Mannucci PM (1974) Clotting factors in von Willebrand’s disease. Lancet 11: 105–10

    Google Scholar 

  • Ruggeri ZM, Mannucci PM, Jeffcoate SL, Ingram GIC (1976) Immunoradiometric assay of factor VHI-related antigen. With observations in 32 patients with von Willebrand’s disease. Br J Haematol 33: 221–23

    PubMed  CAS  Google Scholar 

  • Ruggeri ZM, Mannucci PM, Bader R, Barbui T (1978) Factor VHI-related properties in platelets from patients with von Willebrand’s disease. J Lab Clin Med 91: 132–140

    PubMed  CAS  Google Scholar 

  • Ruggeri ZM, Ciavarella N, Mannucci PM (1979) Familian incidence of precipitating antibodies in von Willebrand’s disease. A study of four cases. J Lab Clin Med 94: 60–75

    Google Scholar 

  • Ruggeri ZM, Pareti FI, Mannucci PM et al. (1980) Heightened interaction between platelets and factor Vlll/von Willebrand factor in a new subtype of von Willebrand’s disease. N Engl J Med 302: 1047–105

    PubMed  CAS  Google Scholar 

  • Ruggeri ZM, Bader R, Coppola R et al. (1981) The multimeric structure of factor Vlll/von Wille-brand factor influences binding to a specific thrombin-induced receptor on the platelet surface. Blood [Suppl 1] 58: 204a

    Google Scholar 

  • Ruggeri ZM, Bader R, Marco L de (1982a) Glanzmann thrombasthenia: deficient binding of von Willebrand factor to thrombin-stimulated platelets. Proc Natl Acad Sci USA 79: 6038–6041

    PubMed  CAS  Google Scholar 

  • Ruggeri ZM, Lombardi R, Gatti L et al. (1982b) Type IIB von Willebrand’s disease: differential clearance of endogenous versus transfused large multimer von Willebrand factor. Blood 60: 1453

    PubMed  CAS  Google Scholar 

  • Ruggeri ZM, Mannucci PM, Federici AB ( 1982 c) Multimeric composition of factor Vlll/von Willebrand factor following administration of DDAVP: Implications for pathophysiology and therapy of von Willebrand’s disease subtypes. Blood 59: 1272–1278

    Google Scholar 

  • Ruggeri ZM, Nilsson IM, Lombardi R et al. (1982d) Aberrant multimeric structure of von Willebrand factor in a new variant of von Willebrand’s disease (type IIC). J Clin Invest 70: 1124–1127

    PubMed  CAS  Google Scholar 

  • Ruggeri ZM, De Marco L, Gatti L et al. (1983) Platelets have more than one binding site for von Willebrand factor. J Clin Invest 72: 1–12

    PubMed  CAS  Google Scholar 

  • Sakariassen KS, Bolhuis PA, Sixma J J (1979) Human blood platelet adhesion to artery subendothelium is mediated by factor VIII/von Willebrand factor bound to the subendothelium. Nature 279: 636–63

    PubMed  CAS  Google Scholar 

  • Sakariassen KS, Ottenhof-Rovers M, Sixma JJ (1984) Factor VIII-von Willebrand factor requires calcium for facilitation of platelets adherence. Blood 63: 996–1003

    PubMed  CAS  Google Scholar 

  • Salzman EW (1963) Measurement of platelet adhesiveness: a simple in vitro technique demonstrating an abnormality in von Willebrand’s disease. J Lab Clin Med 62: 724–735

    PubMed  CAS  Google Scholar 

  • Santoro SA (1981) Adsorption of von Willebrand factor/factor VIII by the genetically distinct interstitial collagens. Thromb Res 21: 689

    PubMed  CAS  Google Scholar 

  • Santoro SA (1983) Preferential binding of high molecular weight forms of von Willebrand factor to fibrillar collagen. Biochem Biophys Acta 756: 123

    PubMed  CAS  Google Scholar 

  • Sarji KE, Stratton RD, Wagner RH, Brinkhous KM (1974) Nature of von Willebrand factor: a new assay and a specific inhibitor. Proc Natl Acad Sei USA 71: 2937

    CAS  Google Scholar 

  • Savidge GF, Carlebjörk G (1979) An optimised radioimmunoassay of F-VIII-related antigen ( FVIII-RAg) in plasma and eluates. Thromb Res 15: 363–376

    Google Scholar 

  • Scharrer I (1979) Bedeutung des Ristocetin-Cofaktors und der Ristocetin-induzierten Plättchenaggre-gation. In: Landbeck G, Marx R, Stolte HP (Hrsg) 10. Haemophilie Symp. Hamburg 1979. Pharmazeutische Verlagsgesellschaft, München, S 57–89

    Google Scholar 

  • Scharrer I (1980) Treatment of von Willebrand’s disease. In: Mammen EF, Barnhart MI, Lusher JM, Walsh RT (eds) Treatment of bleeding disorders. PID Publications Limited, Westbury/NY 11590,p 101

    Google Scholar 

  • Scharrer I (1981) Klinische Anwendung des DDAVP bei zahnärztlichen Eingriffen. In: Sutor AH (ed) Vasopressin Analogues and Haemostasis (2nd Intern Sympos on DDAVP and Glycylpressin in bleeding disorders, Münster 1980 ) Schattauer, Stuttgart New York, p 14

    Google Scholar 

  • Scherer HE, Niehues B, Lechler E (1982) Mitralklappenprolaps bei von Willebrand-Jürgens-Syndrom. In: Loo J van de, Asbeck F (Hrsg) Hämostase, Thrombophilie und Arteriosklerose. (Berichtband 2. Kongreß für Thrombose und Hämostase, Münster 1982). Schattauer, Stuttgart New York, S 511

    Google Scholar 

  • Schimpf K, Rothmann P (1980) Behandlung mit DDAVP während 18 Zahnextraktionen, 2 Muskel-blutungen, 1 Bisswunde und einer Kieferhöhlenspülung bei insgesamt 8 Patienten mit milder Haemophilie A. In: Sutor AH (ed) DDAVP in bleeding disorders (1st Int. Sympos. on DDAVP in bleeding disorders, Münster 1979 ). Schattauer, Stuttgart New York

    Google Scholar 

  • Schmitz-Huebner U, Balleisen L, Arends P, Pollmann H, Sutor AH (1980) DDAVP-induced changes of factor Vlll-related activities and bleeding time in patients with von Willebrand’s syndrome. Haemostasis 9: 204

    PubMed  CAS  Google Scholar 

  • Schneider-Trip MD, Jenkins CSP, Kahle LH (1979) Studies on the mechanism of ristocetin-induced platelet aggregation: Binding of factor VIII to platelets. Br J Haematol 43: 99

    Google Scholar 

  • Scott JP, Montgomery RR (1981) Platelet von Willebrand’s antigen II: active release by aggregating agents and a marker of platelet release reaction in vivo. Blood 58: 1075–1080

    PubMed  CAS  Google Scholar 

  • Scott JP, Montgomery RR, Tubergen DG, Hays T (1981) Acquired von Willebrand’s disease in association with Wilm’s tumor: Regression following treatment. Blood 58: 665–669

    Google Scholar 

  • Shapiro GA, Anderson JC, Pizzo SV, McKee PA (1973) The subunit structure of normal and hemophilic factor VIII. J Clin Invest 52: 2198–2210

    PubMed  CAS  Google Scholar 

  • Shoai I, Lavergne JM, Ardaillou N et al. (1977) Heterogeneity of von Willebrand’s disease. Study of 40 Iranian cases. Br J Haematol 37: 67–83

    CAS  Google Scholar 

  • Silwer J (1973) von Willebrand’s disease in Sweden. Acta Paediatr Scand [Suppl] 238 Silwer N, Cronberg JS, Nilsson IM (1966) Occurrence of ateriosklerosis in von Willebrand’s disease. Acta Med Scand 180: 475–484

    Google Scholar 

  • Simone JV, Cornet JA, Abildgaard CF (1968) Acquired von Willebrand’s syndrome in systemic lupus erythematosis. Blood 31: 806–812

    PubMed  CAS  Google Scholar 

  • Sixma JJ, Kater L, Bouma BN et al. (1976) Immunofluorescent localization of factor Vlll-related antigen, fibrinogen, and several other plasma proteins in hemostatic plugs in humans. J Lab Clin Med 87: 112–11

    PubMed  CAS  Google Scholar 

  • Sixma JJ, Over J, Bouma BN, Bloom AL, Peake IR (1978) Predominance of normal low molecular weight forms or factor VIII in “variant” von Willebrand’s disease. Thromb Res 12: 929–936

    PubMed  CAS  Google Scholar 

  • Sixma JJ, Sakariassen KS, Houdijk W, Aarts PAM (1983) Die Bedeutung der Plättchen-Gefäßwand-Reaktion in der Pathogenese der Atherosklerose. Arzneimittelforsch 33: 1355–1357

    Google Scholar 

  • Sixma JJ, Sakariassen KS, Beeser-Visser NH et al. (1984) Adhesion of platelets to human artery subendothelium: effect of factor VIII-von Willebrand factor of various multimeric composition. Blood 63: 128–13

    PubMed  CAS  Google Scholar 

  • Slot JW, Bouma BN, Montgomery R, Zimmerman TS (1978) Platelet factor VIH-related antigen: Immunofluorescent localization. Thromb Res 13: 871–881

    Google Scholar 

  • Sodetz JM, Pizzo SV, McKee PA (1977) Relationship of sialicacid to function and in vivo survival of human factor VHI-von Willebrand factor protein. J Biol Chem 252: 5538–5546

    PubMed  CAS  Google Scholar 

  • Sodetz JM, Paulson JC, Pizzo SV, McKee PA (1978) Carbohydrate on human factor VHI/von Willebrand factor. Impairment of function by removal of specific galactose residues. J Biol Chem 253: 7202–720

    Google Scholar 

  • Soff GA, Levin J (1981) Familial multiple coagulation factors deficiencies. I. Review of the literature: Differentiation of single hereditary disorders associated with multiple factor deficiencies from coincidental concurrence of single factor deficiency states. Semin Thromb Hemost VII /2: 112

    Google Scholar 

  • Solum NO, Hagen I, Peterka M, Gjemdal T (1979) Absence of the 145000 molecular weight, soluble platelet membrane glycoprotein-lack of agglutination. Thromb Haemostas 42: 1626

    Google Scholar 

  • Solum NO, Hagen I, Filion-Myklebust C, Staback T (1980) Platelet glycocalicin: Its membrane association and solubilization in aqueous media. Biochim Biophys Acta 597: 235

    Google Scholar 

  • Stableforth P, Tamagnini GL, Dormandy KM (1976) Acquired yon Willebrand syndrome with inhibitors both to factor VIII clotting activity and ristocetin-induced platelet aggregation. Br J Haematol 33: 565–57

    PubMed  CAS  Google Scholar 

  • Stel HV, Sakariassen KS, Scholte BJ et al. (1984) Characterization of 25 monoclonal antibodies to factor VIII-von Willebrand factor: relationship between ristocetin-induced platelet aggregation and platelet adherence to subendothelium. Blood 63: 1408–1415

    PubMed  CAS  Google Scholar 

  • Stibbe J (1977) Effect of exercise on FVIII-complex: proportional increase of ristocetin cofactor (von Willebrand factor) and FVIII-AGN, but disproportional increase of FVIII-AHF. Thromb Res 1: 163–16

    Google Scholar 

  • Stibbe J, Kirby EP (1976) The influence of haemaccel, fibrinogen and albumin on ristocetin-induced platelet aggregation. Relevance to the quantitative measurement of the ristocetin cofactor. Thromb Res 8: 151–16

    Google Scholar 

  • Stites DP, Hershgold EP, Perlman JD, Fudenberg HH (1971) Factor VIII detection by hemagglutination inhibition: Hemophilic A and von Willebrand’s disease. Science 171: 196–197

    Google Scholar 

  • Stratton RD, Wagner RH, Webster WP et al. (1975) Antibody nature of circulating inhibitor of plasma of von Willebrand factor. Proc Natl Acad Sci USA 72: 4167–4171

    PubMed  CAS  Google Scholar 

  • Strauss HS, Bloom GE (1965) von Willebrand’s disease. Use of a platelet-adhesiveness test in diagnosis and family investigations. N Engl J Med 273: 171–181

    Google Scholar 

  • Strauss HS, Diamond LK (1963) Elevation of factor VIII (antihemophilic factor) during pregnancy in normal persons and in a patient with von Willebrand’s disease. N Engl J Med 269: 1251

    PubMed  CAS  Google Scholar 

  • Stuart MJ, Miller ML, Davey FR, Walk JA (1979) The postaspirin bleeding time: a screening test for evaluating haemostatic disorders. Br J Haematol 43: 649–659

    PubMed  CAS  Google Scholar 

  • Sultan Y (1981) Rationale for the treatment of von Willebrand’s disease. In Haemophilia and Haemostasis. Liss AR, Inc, New York, pp 149–162

    Google Scholar 

  • Sultan Y, Bernal-Hoyos EJ, Levy-Toledano S, Jeanneau C, Caen JP (1974) Dominant inherited familial factor VIII deficiency (von Willebrand disease) associated with thrombo-cytopathic thrombocytopenia (biologic and genetic implications). Pathol Biol 22: 27

    PubMed  Google Scholar 

  • Sultan Y, Simeon J, Caen JP (1975) Detection of heterozygotes in both parents of homozygous patients with von Willebrand’s disease. J Clin Pathol 28: 309–316

    PubMed  CAS  Google Scholar 

  • Sultan Y, Simeon J, Caen JP (1976) Electrophoretic heterogeneity of normal factor VHI/von Willebrand protein, and abnormal electrophoretic mobility in patients with von Willebrand’s disease. J Lab Clin Med 87: 185–197

    PubMed  CAS  Google Scholar 

  • Sultan Y, Bouma BN, Graaf S de, Simeon J, Caen JP, Sixma JJ (1977) Factor VIII related antigen in platelets of patients with von Willebrand’s disease. Thromb Res 11: 23–30

    PubMed  CAS  Google Scholar 

  • Sultan Y, Jeanneau C, Lamaziere J, Maisonneuve P, Caen JP (1978) Platelet factor VIH-related antigen: Studies in vivo after transfusion in patients with von Willebrand disease. Blood 51: 751–76

    Google Scholar 

  • Sultan Y, Maisonneuve P, Angeles-Cano E (1979) Release of VIIIR:Ag and VIIIR:WF during thrombin and collagen induced aggregation. Thromb Res 15: 415–425

    PubMed  CAS  Google Scholar 

  • Sussman II, Weiss HJ (1978) Dissociation of factor VIII in the presence of proteolytic inhibitors. Thromb Haemostas 40: 316–32

    CAS  Google Scholar 

  • Sutor AH, Bowie EJW, Owen CA Jr (1971 a) A new method of demonstrating the effect of therapy in von Willebrand’s disease. Mayo Clin Proc 46: 345–346

    Google Scholar 

  • Sutor AH, Bowie EJW, Thompson JH, Didisheim P, Mertens BF, Owen CA (1971b) Bleeding from standardized skin punctures: automated technic for recording time, intersity and pattern of bleeding. Am J Clin Pathol 55: 541

    PubMed  CAS  Google Scholar 

  • Sutor AH, Pollmann H, Arends P, Schmitz-Huebner U, Balleisen L (1980) DDAVP-induzierte Ver-änderungen der primären Haemostase bei Patienten mit von Willebrand-Jürgens-Syndrom. In: Sutor AH (ed) DDAVP in bleeding disorders (1st Intern Sympos on DDAVP in bleeding disorders Münster 1979 ). Schattauer, Stuttgart New York, p 85

    Google Scholar 

  • Switzer ME, McKee PA (1976) Studies on human antihemophilic factor. Evidence for a covalently linked subunit structure. J Clin Invest 57: 925–937

    Google Scholar 

  • Takahashi H (1980) Studies on the pathophysiology and treatment of von Willebrand’s disease: IV. Mechanism of increased ristocetin-induced platelet aggregation in von Willebrand’s disease. Thromb Res 19: 857–86

    PubMed  CAS  Google Scholar 

  • Takahashi H (1981) Studies on the pathophysiology and treatment of von Willebrand’s disease. V.Properties of factor VIII after DDAVP infusion in variant von Willebrand’s disease. Thromb Res 21: 357–36

    PubMed  CAS  Google Scholar 

  • Takahashi H (1984) Type lib and platelet-type von Willebrand’s disease. Heightened interaction between von Willebrand factor and platelets. Blood Vessel 15: 111–127

    Google Scholar 

  • Takahashi H, Sakuragawa N, Shibata A (1980) von Willebrand disease with an increased ristocetin-induced platelet aggregation and a qualitative abnormality of the factor VIII protein. Am J Hematol 8: 299–30

    Google Scholar 

  • Taylor J (1968) von Willebrand’s disease in obstetrics and gynaecology. J Obstet Gynaecol Br Commonw 75:453

    Google Scholar 

  • Telfer MC, Chediak J (1977) Factor VHI-related disorders and their relationship to pregnancy. JReprod Med 19: 211

    CAS  Google Scholar 

  • Theiss W, Sauer E (1977) DDAVP: Alternative to replacement treatment in mild haemophilia A and von Willebrand-Jürgens syndrome. Dtsch Med Wochenschr 102: 1769–1772

    Google Scholar 

  • Theiss W, Schmidt G (1978) DDAVP in von Willebrand’s disease: repeated administration and the behaviour of the bleeding time. Thromb Res 13: 1119

    PubMed  CAS  Google Scholar 

  • Thomson C, Forbes CD, Prentice CRM (1973) Relationship of factor VIII to ristocetin induced platelet aggregation. Effect of heterologous and acquired factor VIII antibodies. Thromb Res 3: 363

    Google Scholar 

  • Tilsner V, Reuter H (1982) Nebenwirkungen der Faktor Vlll-Substitution bei Patienten mit Hämophilie A. Münchner Med Wochenschr 124: 553

    CAS  Google Scholar 

  • Ts’ao CH, Green D, Schultz K (1976) Function and ultrastructure of platelets of neonates: enhanced ristocetin induced aggregation of neonatal platelets. Br J Haematol 32: 225–233

    PubMed  Google Scholar 

  • Tschopp TB, Weiss HJ, Baumgartner HR (1974) Decreased adhesion of platelets to subendothelium in von Willebrand’s disease. J Lab Clin Med 83: 296–300

    PubMed  CAS  Google Scholar 

  • Tschopp TB, Baumgartner HR, Silberbauer K, Sinzinger H (1979) Platelet adhesion and platelet thrombosis formation on subendothelium of human arteries and veins exposed to flowing blood in vitro. A comparison with rabbit aorta. Haemostasis 8: 19–29

    Google Scholar 

  • Tuddenham EGD, Trabold NC, Collins JA, Hoyer LW (1979) The properties of factor VIII coagulant activity prepared by immunoadsorbent chromatography. J Lab Clin Med 93: 40–53

    PubMed  CAS  Google Scholar 

  • Tuddenham EGD, Lazarchick J, Hoyer LW (1981) Synthesis and release of factor VIII by cultured human endothelial cells. Br J Haematol 47: 617–626

    PubMed  CAS  Google Scholar 

  • Tuddenham EGD, Lane RS, Rotblat F et al. (1982) Response to infusions of polyelectrolyte fractionated human factor VIII concentrate in human haemophilia A and von Willebrand’s disease. Br J Haematol 52: 259–267

    PubMed  CAS  Google Scholar 

  • Turitto VT, Baumgartner HR (1983) Platelet adhesion. In: Harker LA, Zimmerman ThS (eds) Measurements of platelet function, methods in hematology, vol 8. Churchill Livingstone, Edinburgh London Melbourne New York, pp 46–63

    Google Scholar 

  • Turitto V, Weiss H, Sussman I, Zimmerman T (1981) Factor VIII in vessel wall influences platelet interaction with subendothelium. Thromb Haemostas 46: 199

    Google Scholar 

  • Valente A, Volpe E, Gandini M, Buonanna G (1972) von Willebrand’s disease: platelet nucleotide alterations in a case with a marked platelet adhesiveness and aggregation defects. Acta Haematol 47: 182

    Google Scholar 

  • Vehar GA, Davie EW (1980) Preparation and properties of bovine factor VIII (antihemophilic factor). Biochemistry 19: 401–410

    PubMed  CAS  Google Scholar 

  • Veitkamp JJ, Tilburg NH von (1973) Detection of heterozygotes for recessive von Willebrand’s disease by the assay of antihemophilic factor like antigen. N Engl J Med 289: 882–885

    Google Scholar 

  • Veltkamp JJ, Stevens P, Pias M van de et al. (1970) Production site of bleeding factor (acquired morbus von Willebrand). Thromb Diath Haemorrh 23: 412

    PubMed  CAS  Google Scholar 

  • Vermylen J, Donati MD, Gaetano G de et al. (1973) Aggregation of human platelets by bovine or human factor VIII: role of carbohydrate side chains. Nature 244: 167–168

    PubMed  CAS  Google Scholar 

  • Vermylen J, Gaetano G de, Donati MB et al. (1974) Platelet-aggregating activity in neuraminidase treated human cryoprezipitates: its correlation with factor VHI-related antigen. Br J Haematol 26: 645–65

    PubMed  CAS  Google Scholar 

  • Vermylen J, Bottecchia D, Szpilman H (1976) Factor VIII and human platelet aggregation. III. Further studies on aggregation of human platelets by neuraminidase-treated human factor VIII. Br J Haematol 34: 321–330

    Google Scholar 

  • Vilhardt H, Äberg M, Nilsson IM (1980) Possible uses of DDAVP in coagulation disorders. In: Sutor AH (ed) DDAVP in bleeding disorders. (1st Intern Symposium on DDAVP in bleeding disorders, Münster 1979 ). Schattauer, Stuttgart New York, p 36

    Google Scholar 

  • Voss H von (1982) Bestimmungsmethoden des Faktor VIII:RCF. In: Landbeck G, Marx R, Stolte HP (Hrsg) 11. Haemophilie Symp Hamburg 1980. Pharmazeutische Verlagsgesellschaft, München, S 81–9

    Google Scholar 

  • Vukovich TH, Koller E, Doleschel W, Mayr WR (1980) Faktor VHI-Präparate unterschiedlicher Reinheit-Untersuchung physiko-chemischer Parameter. Wien Klin Wochenschr 92: 385

    PubMed  CAS  Google Scholar 

  • Wagner DD, Marder VJ (1983) Biosynthesis of von Willebrand protein by human endothelial cells: Identification of a large precursor polypeptide chain. J Biol Chem 258: 2065

    Google Scholar 

  • Wahlberg TB, Blomback M, Ruggeri ZM (1983) Differences between heterozygous dominant and recessive von Willebrand’s disease type I expressed by bleeding symptoms and combinations of factor VIII variables. Thromb Haemostas 50: 864–868

    CAS  Google Scholar 

  • Walker EH, Dormandy KM (1968) The management of pregnancy in von Willebrand’s disease. J Obstet Gynaec Br Cwlth 75: 459

    CAS  Google Scholar 

  • Wall RT, Counts RB, Harker LA, Striker GE (1980) Binding and release of factor VIII/von Willebrand factor by human endothelial cells. Br J Haematol 46: 287–298

    PubMed  CAS  Google Scholar 

  • Warner AI, Lusher JM (1983) DDAVP a useful alternative to blood components in moderate haemophilia A and in von Willebrand’s disease. J Pediatr 102: 228

    Google Scholar 

  • Wautier JL, Levy-Toledano S, Caen JP (1976) Acquired von Willebrand’s syndrome and thrombopathy in a patient with chronic lymphocytic leukemia. Scand J Haematol 16: 128

    PubMed  CAS  Google Scholar 

  • Weinger RS, Cimo PL, Moake JL (1981) Type IIB von Willebrand’s disease: Unusual response to cryoprecipitate infusion. Ann Intern Med 94: 47–50

    Google Scholar 

  • Weinstein M, Deykin D (1979) Comparison of factor VHI-related von Willebrand factor proteins prepared from human cryoprecipitate and factor VIII concentrate. Blood 53: 1095–1105

    PubMed  CAS  Google Scholar 

  • Weinstein M, Chute L, Deykin D (1981) Analysis of factor VIII coagulant antigen in normal, thrombin-treated, and hemophilic plasma. Proc Natl Acad Sei USA 78: 5137–5141

    CAS  Google Scholar 

  • Weiss HJ (1974) Relation of von Willebrand factor to bleeding time. N Engl J Med 291: 420

    PubMed  CAS  Google Scholar 

  • Weiss HJ (1975) Abnormalities of factor VIII and platelet aggregation-use of ristocetin in diagnosing the von Willebrand syndrome. Blood 45: 403–412

    PubMed  CAS  Google Scholar 

  • Weiss HJ (1977) von Willebrand’s disease. In: Williams WJ, Beutler E, Ersley EJ, Rundles RW (eds) Hematology. McGraw-Hill, New York, p 1434

    Google Scholar 

  • Weiss HJ, Hoyer LW (1973) von Willebrand factor: Dissociation from antihemophilic factor procoagulant activity. Science 182: 1149–1151

    Google Scholar 

  • Weiss HJ, Kochwa S (1970) Molecular forms of antihaemophilic globulin in plasma, cryoprecipitate and after thrombin activation. Br J Haematol 18: 89–100

    PubMed  CAS  Google Scholar 

  • Weiss HJ, Rogers J (1972) Correction of the platelet abnormality in von Willebrand’s disease by cryoprecipitate. Am J Med 53: 734

    PubMed  CAS  Google Scholar 

  • Weiss HJ, Phillips LL, Rosner W (1972) Separation of subunits of antihemophilic factor ( AHR) by agarose gel chromatography. Thromb Diath Haemorrh 27: 212–219

    Google Scholar 

  • Weiss HJ, Hoyer LW, Rickles FR, Varma A, Rogers J (1973a) Quantitative assay of a plasma factor deficient in von Willebrand’s disease that is necessary for platelet aggregation. Relationship to factor VIII procoagulant activity and antigen content. J Clin Invest 52: 2708–271

    Google Scholar 

  • Weiss HJ, Rogers J, Brand H (1973 b) Defective ristocetin-induced platelet aggregation in von Willebrand’s disease and its correction by factor VIII. J Clin Invest 52: 2697–2707

    Google Scholar 

  • Weiss HJ, Tschopp TB, Baumgartner HR (1974) Decreased adhesion of giant (Bernard-Soulier) platelets to subendothelium. Further implications on the role of the von Willebrand factor in hemostasis. Am J Med 57: 920–925

    Google Scholar 

  • Weiss HJ, Sussmann II, Hoyer LW (1977) Stabilization of factor VIII in plasma by the von Willebrand factor. J Clin Invest 60: 390–404

    PubMed  CAS  Google Scholar 

  • Weiss HJ, Baumgartner HR, Tschopp TB (1978 a) Correction by factor VIII of the impaired platelet adhesion to subendothelium in von Willebrand’s disease. Blood 51: 267–279

    Google Scholar 

  • Weiss HJ, Turitto VT, Baumgartner HR ( 1978 b) Effect of shear rate on platelet interaction with subendothelium in citrated and native blood. I. Shear dependent decrease of adhesion in von Willebrand’s disease and the Bernard-Soulier syndrome. J Lab Clin Med 92: 750–764

    Google Scholar 

  • Weiss HJ, Ball AP, Mannucci PM (1982 a) Incidence of severe von Willebrand’s disease. N Engl J Med 307:127 (letter)

    Google Scholar 

  • Weiss HJ, Meyer D, Rabinowitz R (1982b) Pseudo von Willebrand’s disease: An intrinsic platelet defect with aggregation by unmodified human factor VIII/von Willebrand factor and enhanced adsorption of its high molecular weight multimers. N Engl J Med 306: 326–333

    Google Scholar 

  • WHO Expert Committee (1977) Methods for the detection of haemophilia carriers: A memorandum. Bull WHO 55: 675

    Google Scholar 

  • Willebrand EA von (1926) Hereditär pseudohamofili. Finska laeksaellsk handl 68: 87–112

    Google Scholar 

  • Willebrand EA von (1931) Ueber hereditaere Pseudohaemophilie. Acta Med Scand 76: 521–550

    Google Scholar 

  • Willebrand EA von, Jürgens R (1933 a) Über eine neue Bluterkrankheit, die konstitutionelle Thrombopathie. Klin Wochenschr 12: 414

    Google Scholar 

  • Willebrand EA von, Jürgens R (1933b) Über ein neues und vererbbares Blutungsübel: Die konstitutionelle Thrombopathie. Dtsch Arch Klin Med 175: 453

    Google Scholar 

  • Winckelmann G, Böttcher D (1980) Die Ristocetin induzierte Plättchenaggregation in der Diagnostik des von Willebrand Syndroms. In: Voss H von, Göbel U (Hrsg) Praktische Anwendung der Thrombozytenfunktionsdiagnostik. Thieme, Stuttgart, S 151

    Google Scholar 

  • Winckelmann G, Groh R, Schneider J, Huber P (1967) Schwangerschaft und Entbindung bei Patientinnen mit einem von Willebrand-Jürgens-Syndrom. Verhalten des Faktor VIII (antihämophilen Globulin). Dtsch Med Wochenschr 92: 436

    Google Scholar 

  • Zettervall O, Nilsson IM (1978) Acquired von Willebrand’s disease caused by monoclonal antibody. Acta Med Scand 204: 521

    PubMed  CAS  Google Scholar 

  • Zimmerman TS, Roberts JR (1980) Factor VHI-related antigen, In: Nakamura RM, Dito WR, Tucker ES III (eds) Immunoassays. Clinical laboratory techniques for the 1980s. Alan R Liss, New York

    Google Scholar 

  • Zimmerman TS, Ruggeri ZM (1982) Von Willebrand’s disease. Prog Hemost Thromb 6: 203–236

    PubMed  CAS  Google Scholar 

  • Zimmerman TS, Ruggeri ZM (1983) Von Willebrand’s disease. Clin Haematol 12: 175–200

    PubMed  CAS  Google Scholar 

  • Zimmerman TS, Ratnoff OD, Powell AE (1971) Immunologic differentiation of classic hemophilia (factor VIII deficiency) and von Willebrand’s disease. J Clin Invest 50: 244 — 254

    PubMed  CAS  Google Scholar 

  • Zimmerman TS, Hoyer LW, Dickson L, Edgington TS (1975 a) Determination of the von Willebrand’s disease antigen (factor VHI-related antigen) in plasma by quantitative immunoelectrophoresis. J Lab Clin Med 86: 152–159

    Google Scholar 

  • Zimmerman TS, Roberts J, Edgington TS ( 1975 b) Factor VHI-related antigen. Multiple molecular forms in human plasma. Proc Natl Acad Sei USA 72: 5121–5125

    Google Scholar 

  • Zimmerman TS, Abildgaard C, Meyer D (1979 a) The factor VIII abnormality in severe von Willebrand’s disease. N Engl J Med 301: 1307–1310

    Google Scholar 

  • Zimmerman TS, Voss R, Edgington TS (1979b) Carbohydrate of factor VIII/von Willebrand factor in von Willebrand’s disease. J Clin Invest 64: 1298–1302

    PubMed  CAS  Google Scholar 

  • Zimmerman TS, Roberts JR, Ruggeri ZM (1982) Factor VHI-related antigen: Characterization by electrophoretic techniques. In: Bloom AL (ed) The hemophilias, methods in hematology, vol 5. Churchill Livingstone, Edinburgh London Melbourne New York, pp 81–91

    Google Scholar 

  • Zucker MB, Kim SJA, McPherson J, Grant RA (1977) Binding of factor VIII to platelets in the presence of ristocetin. Br J Haematol 35: 535

    PubMed  CAS  Google Scholar 

  • Zucker MB, Broeckman MJ, Kaplan KL (1979) Factor VHI-related antigen in human blood platelets. Localization and release by thrombin and collagen. J Lab Clin Med 94: 675–682

    Google Scholar 

  • Zuzel M, Nilsson IM, Aberg M (1978) A method for measuring plasma ristocetin cofactor activity. Thromb Res 12: 745–75

    PubMed  CAS  Google Scholar 

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  1. H. Niessner
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  1. I. Medizinische Klinik, Klinikum Mannheim Fakultät für Klinische Medizin Mannheim, Universität Heidelberg, Theodor-Kutzer-Ufer, D-6800, Mannheim 1, Germany

    Dieter L. Heene

  2. Orthopädische Abteilung/Krankenhaus, A-2700, Wiener Neustadt, Austria

    P. Bösch

  3. I. Medizinische Universitätsklinik, Röntgenstation, Lazarettgasse 14, A-1090, Wien, Austria

    H. Czembirek

  4. I. Medizinische Universitätsklinik, Lazarettgasse 14, A-1090, Wien, Austria

    E. Deutsch , K. Lechner  & H. Niessner ,  & 

  5. I. Medizinische Universitätsklinik, Röntgenstation 9, Lazarettgasse 14, A-1090, Wien, Austria

    J. Haller

  6. Universitäts-Kinderklinik, Klinikum der Universität, Mathildenstr. 1, D-7800, Freiburg i. Brsg., Germany

    W. Künzer  & H. Niederhoff (Akademischer Oberrat) &  (Akademischer Oberrat)

  7. School of Medicine, Department of Surgery 6-C, University Health Center, Wayne State University, 4201 St. Antoine, 48201, Detroit, MI, USA

    E. F. Mammen

  8. Klinik für Innere Medizin, Zentralkrankenhaus St. Jürgen-Str., D-2800, Bremen, Germany

    H. Rasche

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Niessner, H. (1985). Das v. Willebrand-Jürgens-Syndrom. In: Heene, D.L., et al. Blut und Blutkrankheiten. Handbuch der Inneren Medizin, vol 2 / 9. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-70514-4_5

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