Abstract
Platelet von Willebrand Factor (vWF) was studied in normal donors (n = 10) and in patients with von Willebrand disease (vWD) type I (n = 12). The vWF antigen (Ag) and the functional parameter, measured as the vWF collagen binding activity (CBA), were determined by parallel quantitative ELISAs. In the platelets of normal donors the concentrations (mean±SD) of Ag and CBA were 0.40 ±0.18 U/109 platelets and 0.44 ± 0.24 U/109 platelets, respectively, with a CBA to Ag ratio of 1.06 ±0.17. There was no correlation between the plasma concentrations of Ag and CBA with those in platelets either in normal donors or in patients with vWD type I. On the basis of their concentrations of Ag and CBA in platelets, patients could be subdivided into two groups: (1) platelet normal, i.e. those with a normal Ag and CBA content of 0.35±0.18 U/109 platelets and 0.42±0.21 U/109 platelets, respectively, and (2) platelet low/discordant, i.e. those with a low Ag and CBA content of 0.15±0.06U/109 platelets and 0.07±0.03 U/109 platelets, respectively. Patients with “platelet low/discordant” vWF content had an increased bleeding tendency when compared to those patients with “platelet normal”, confirming the relevance of platelet vWF in haemostasis.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
References
Ginsburg D, Bowie EJW (1992) Molecular genetics of von Willebrand disease. Blood 79: 2507–2519
Meyer D, Girma JP (1993) Von Willebrand factor: structure and function. Thromb Haemostas 70: 99–103
Werner EJ, Broxson EH, Tucker EL, Giroux DS, Shults J, Abshire TC (1993) Prevalence of von Willebrand disease in children, a multiethnic study. J Pediatr 123: 893–898
Eikenboom JCJ, Reitsma PH, Peerlinck KM J, Briet E (1993) Recessive inheritance of von Willebrand’s disease. Lancet 341: 982–986
Howard MA, Firkin BG (1971) Ristocetin — a new tool in the investigation of platelet aggregation. Thromb Diath Haemorrh 26: 362–369
Favaloro EJ, Grispo L, Exner T, Koutts J (1991) Development of a simple collagen based ELISA assay aids in the diagnosis of, and permits sensitive discrimination between type I and type II, von Willebrand’s disease. Blood Coag Fibrinol 2: 285–291
Choong SC, Thomas KB; Tune EP, Collicut M, Firkin BG (1992) Analysis of human von Willebrand factor (vWF) in a routine laboratory. 24th Congress of the International Society of Haematology (abstr 490)
Thomas KB, Choong SC, Jessat U, Zieger B, Budde U, Tune EP, Grohmann A, Sutor AH (1993) Preliminary results of von Willebrand factor — collagen binding activity and ristocetin cofactor activity. A comparison of methods able to differentiate von Willebrand disease type I and II. In: Scharrer I, Schramm W (eds) 24. Hamophilie-Symposion, Hamburg 1993. Springer, Berlin Heidelberg New York
Ruggeri ZM, Zimmerman TS (1987) Von Willebrand factor and von Willebrand disease. Blood 70: 895–904
Federici AB, de Groot PG, Moia M, Ijsseldijk MJW, Sixma JJ, Mannucci PM (1993) Type I von Willebrand disease, subtype ‘platelet low’: decreased platelet adhesion can be explained by low synthesis of von Willebrand factor in endothelial cells. Br J Haematol 83: 88–93
Howard MA, Montgomery DC, Hardisty RM (1974) Factor-VIII-related-antigen in platelets. Thromb Res 4: 617–624
Koutts J, Walsh PN, Plow EF, Fenton J, Bouma BN, Zimmerman TS (1978) Active release of human platelet factor VHI-related antigen by adenosine diphosphate, collagen and thrombin. J Clin Invest 62: 1255–1263
Fernandez FL, Ginsberg MH, Ruggeri ZM, Battle FJ; Zimmerman TS (1982) Multimeric structure of platelet factor VHI/von Willebrand factor: the presence of larger multimers and their reassociation with thrombin stimulated platelets. Blood 60: 1132–1138
Thomas KB; Firkin BG, Howard MA (1987) Analysis of platelet von Willebrand factor antigen. Haemostasis 17: 217–225
Bowie EJW, Solberg LA, Fass DN et al (1986) Transplantation of normal bone marrow into pig with severe von Willebrand’s disease. J Clin Invest 78: 26–30
Ware RE, Parker RI, McKeown LP, Graham M (1993) A human chimera for von Wille-brand disease following bone marrow transplantation. Am J Pediatric Hematol Oncol 15: 338–342
Blomback M (1992) von Willebrand’s disease in Scandinavia and on difficulties in diag-nosing von Willebrand’s disease type I. Haemostasis 22:236–244
Mannucci PM; Canciani MT, Rota L, Donovan BS (1981) Response of factor VHI/von Willebrand factor to DDAVP in healthy subjects and in patients with haemophilia A and von Willebrand’s disease. Br J Haematol 47: 283–293
Mannucci PM, Lombardi R, Bader R, Vianello L, Federici AB, Solinas S, Mazzucconi MG, Mariani G (1985) Heterogeneity of type I von Willebrand disease: evidence for a subgroup with an abnormal von Willebrand factor. Blood 66: 796–802
Gralnick HR, Rick ME, McKeown LP et al (1986) Platelet von Willeband factor: an important determinant of the bleeding time in type I von Willebrand’s disease. Blood 68: 58–61
George JN, Thoi LL, Morgan RK (1981) Quantitative analysis of platelet membrane glycoproteins: effect of platelet washing procedures and isolation of platelet density sub-population. Thromb Res 23: 69–77
Raines G, Aumann H, Sykes S, Street A (1990) Multimeric analysis of von Willebrand factor by molecular sieving electrophoresis in sodium dodecyl sulphate agarose gel. Thromb Res 60: 201–212
Gralnick HR, Williams SB, McKeown LP, Magruder L, Hansmann K, Vail M (1991) Platelet von Willebrand factor. Mayo Clin Proc 66: 634–640
McKeown LP, Williams SB, Shafer B, Murray N, Gralnick HR (1993) Alpha granule proteins in type I von Willebrand’s disease. Am J Hematol 42: 158–161
Rodeghiero F, Castaman G, Ruggeri M, Tosetto R (1992) The bleeding time in normal subjects is mainly determined by platelet von Willebrand factor and is independent from the blood group. Thromb Res 65: 605–615
Hashemi S, Tackaberry ES, Palmer DS, Rock G, Ganz PR (1990) DDAVP induces re-lease of von Willebrand factor from endothelial cells in vitro: the effect of plasma and blood cells. Biochim Biophys Acta 1052: 63–70
Boda Z, Pfliegler G,Harsfalvi J, Rak K (1991) Treatment of the severe bleeding episodes in type III von Willebrand’s disease by simultaneous administration of cryoprecipitate and platelet concentrate. Blood Coag Fibrinol 2: 775–777
Castillo R, Escolar G, Monteagudo J, Reverter JC, Ordinas A (1992) Hemostatic effect of platelet von Willebrand factor. Haemostasis 22: 233–235
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 1994 Springer-Verlag Berlin Heidelberg
About this paper
Cite this paper
Thomas, K.B., Zieger, B., Jessat, U., Zehenter, A., Grohmann, A., Sutor, A.H. (1994). Platelet von Willebrand Factor in Patients with von Willebrand Disease Type I: Preliminary Report. In: Scharrer, I., Schramm, W. (eds) 24. Hämophilie-Symposion. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-79009-6_39
Download citation
DOI: https://doi.org/10.1007/978-3-642-79009-6_39
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-540-57999-1
Online ISBN: 978-3-642-79009-6
eBook Packages: Springer Book Archive