Treatment of the haemophilias

  • C. A. Ludlam


Prompt treatment, or prophylaxis, with blood products allows the majority of patients with haemophilia A and B the prospect of leading relatively normal lives. Along with von Willebrand’s disease (vWD), these disorders are numerically and clinically the most important congenital bleeding conditions. The prevelence of haemophilia A is approximately five per hundred thousand of population whereas vWD is probably about half this and haemophilia B (Christmas disease) one fifth; other congenital coagulation deficiencies are rare. Although clinically severe congenital platelet disorders are uncommon, it is becoming increasingly acknowledged that minor abnormalities of platelet function may be relatively common and on occasion clinically important.


Factor VIII Tranexamic Acid Severe Haemophilia Factor Viii Level Factor Viii Inhibitor 
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  1. 1.
    Bloom AL: Inherited Disorders of Blood Coagulation. In: Haemostasis and Thrombosis, Bloom AL, Thomas DP, (eds). Churchill Livingston, 1981; 321–70.Google Scholar
  2. 2.
    Arnold WD, Hilgartner MW: Haemophilic arthropathy. J Bone Joint Surg, 1977; 59: 287–305.PubMedGoogle Scholar
  3. 3.
    Rizza CR: Management of patients with inherited blood coagulation defects. In: Haemostasis and Thrombosis, Bloom AL, Thomas DP (eds). Churchill Livingston, 1981; 371–94.Google Scholar
  4. 4.
    Aronstram A, Choudhury DP, Wassef M, Turk PM, McLellan DS: Double blind controlled trial of three dosage regimens in treatment of haemarthroses in haemophilia A. Lancet, 1980; is 169–71.Google Scholar
  5. 5.
    Allain JP: Dose requirement for replacement therapy in haemophilia A. Thrombosis and Haemostasis, 1979; 42: 825–31.PubMedGoogle Scholar
  6. 6.
    Aronstam A, Wassef M, Hamad Z: Low doses of factor VIII for selected ankle bleeds in severe haemophilia A. Br Med J, 1982; 284: 790.CrossRefGoogle Scholar
  7. 7.
    Small M, Rose PE, MacMillan N, Belch J, Rolfe EB, Forbes CD, Stuart J: Haemophilia and the kidney assessment after 11 year follow-up. Br Med J, 1982; 285: 1609–11.CrossRefGoogle Scholar
  8. 8.
    Jones P, Fearns M, Forbes C, Stuart J: Haemophilia A home therapy in the United Kingdom 1975–7. Br Med J, 1978; is 1447–50.Google Scholar
  9. 9.
    Aronstam A, McLellan DS, Turk P: Transfusion requirements of adolescents with severe haemophilia A. J Clin Path, 1979; 32: 927–30.PubMedCrossRefGoogle Scholar
  10. 10.
    Duthie RB, Matthews JM, Rizza CR, Steel WM: The Management of Musculoskeletal Problems in the Haemophiliacs. Blackwell Scientific Publications 1972.Google Scholar
  11. 11.
    Walsh PN, Rizza CR, Mathews JM, Eipe J, Kernoff PBA, Coles MD, Bloom AL, Kaufman BM, Beck P, Hanan CM, Biggs R: Epsilon-amino caproic acid therapy for dental extraction in haemophilia and Christmas disease: a double blind trial. Br J Haematol, 1971; 20: 463–75.PubMedCrossRefGoogle Scholar
  12. 12.
    Pool JG, Shannon AE: Production of high potency anti-haemophilic factor concentrate prepared from cryoglobulin precipitate. Nature, 1971; 203: 312.CrossRefGoogle Scholar
  13. 13.
    Mason EC: Thaw-siphon technique for production of cryoprecipitate concentrate of factor VIII. Lancet, 1981; ii: 15–17.Google Scholar
  14. 14.
    Smit Sibinga CTh, Welbergen H, Das PC, Griffin B: High yield method of production of freeze-dried purified factor VIII by blood banks. Lancet, 1981; ii: 61–62.Google Scholar
  15. 15.
    Rickard KA, Batey RG, Dovity P, Johnston S, Campbell J, Hodgson J: Hepatitis and Haemophilia Therapy in Australie. Lancet, 1982; ii: 146–40.Google Scholar
  16. 16.
    Stirling ML, Murray J, MacKay P, Black SH, Peutherer JF, Ludlam CA: Incidence of infection with hepatitis B virus in 56 patients with haemophilia A, 1971–1979. J Clin Path, 1983; 36: 577–80PubMedCrossRefGoogle Scholar
  17. 17.
    Mannucci PM, Ruggeri ZM, Pareti FI, Capitanio A: Deamino-8-D-argenine vasopressin; a new pharmacological approach to the management of haemophilia and von Willebrand’s disease. Lancet, 1977; is 869.Google Scholar
  18. 18.
    Bamber M, Murray A, Arborgh BA, Scheuer PJ, Kernoff PB, Thomas HC, Sherlock S: Short incubation non A non B hepatitis transmitted by factor VIII concentrates in patients with congenital coagulation disorders. Gut, 1981; 22: 854–9.PubMedCrossRefGoogle Scholar
  19. 19.
    Szmuness W, Stevens CE, Harley EJ, Zang EA, Oleszko WR, William DC, Sadovsky R, Morrison JM, Kellner A: Hepatitis B vaccine. Demonstration of efficacy in a control led clinical trial in a high-risk population in the United States. N Eng J Med, 1980; 303: 833–941.CrossRefGoogle Scholar
  20. 20.
    Preston FE, Triger DR, Underwood JCE, Bardhan G, Mitchell VE, Stewart RM, Blackburn EK: Percutaneous liver biopsy and chronic liver disease in haemophiliacs. Lancet, 1978; ii: 592–94.Google Scholar
  21. 21.
    Spero JA, Lewis JH, Van Thiel DH, Hasiba U, Rabin BS: Asymptomatic structural liver disease in haemophilia. N Engl J Med, 1978; 298: 1373–78.PubMedCrossRefGoogle Scholar
  22. 22.
    Macek C: Acquired immunodeficiency syndrome cause still elusive. JAMA, 1982; 248: 1423–31.PubMedCrossRefGoogle Scholar
  23. 23.
    Ragni MV, Lewis JH, Spero JA, Bontempo FA: Acquired immunodeficiency-like syndrome in two haemophiliacs. Lancet, 1983; is 213–14.Google Scholar
  24. 24.
    Menitove JE, Aster RH, Casper JT, Lauer SJ, Gottschall JL, Williams JE, Gill JC, Wheeler DV, Piaskowski V, Kirchner P, Montgomery RR: T-Lymphocyte subpopulations in patients with classic haemophilia treated with cryoprecipitate and lyophilised concentrates. N Eng J Med, 1982; 308: 79–83.Google Scholar
  25. 25.
    Lederman MM, Ratnoff OD, Scillian JJ, Jones PK, Schacter B: Impaired cell-mediated immunity in patients with classic haemophilia. N Engl J Med, 1982; 308: 79–83.CrossRefGoogle Scholar
  26. 26.
    Allain JP, Gaillandre A, Lee H: Immunochemical characterization of antibodies to factor VIII in hemophilic and nonhemophilic patients. J Lab Clin Med, 1981; 97: 791–800.PubMedGoogle Scholar
  27. 27.
    Green D, Lechner K: A survey of 215 non-hemophilic patients with inhibitors to factor VIII. Thrombosis and Haemostasis, 1981; 45: 200–208.PubMedGoogle Scholar
  28. 28.
    Wensley RT, Stevens RF, Burn AM, Delamore IW: Plasma exchange and human factor VIII concentrate in managing haemophilia A with factor VIII inhibitors. Br Med J, 1980; 281: 138–9.CrossRefGoogle Scholar
  29. 29.
    Slocombe GW, Newland AC, Colvin MP, Colvin BT: The Role of Intensive plasma exchange in the prevention and management of haemorrhage in patients with inhibitors to factor VIII. Br J Haematol, 1981; 47: 577–85.PubMedCrossRefGoogle Scholar
  30. 30.
    Kernoff PBA, Thomas ND, Lilley PA, Tuddenham EGD: Clinical experience with polyelectrolyte-fractionated porcine factor VIII concentrate. Br J Haematol, 1981; 49: 131.Google Scholar
  31. 31.
    Sjamsoedin LJM, Heijnen L, Nauser-Bunschoten EP, Van Geijiswijk JL, Van Houwelingen H, Van Asten P, Sixma JJ: The effect of activated prothrombin-complex concentrate (FEIBA) on joint and muscle bleeding in patients with haemophilia A and antibodies to factor VIII. N Eng J Med, 1981; 305: 717–21.CrossRefGoogle Scholar
  32. 32.
    Abildgaard CF, Penner JA, Watson-Williams EJ: Antiinhibitor coagulant complex (autoplex) for treatment of factor VIII inhibitors in haemophilia. Blood, 1980; 56: 97884.Google Scholar
  33. 33.
    Hanna WT, Madigan RR, Miles MA, Lange RD: Activated factor IX complex in treatment of surgical cases of haemophilia A with inhibitors. Thrombosis Haemostasis, 1981; 46: 638–41.Google Scholar
  34. 34.
    Bloom AL: Factor VIII inhibitors revisited. Br J Haematol, 1981; 49: 319–24.PubMedCrossRefGoogle Scholar
  35. 35.
    Barrowcliffe TW, Kemball-Cook G, Gray E: Factor VIII Inhibitor bypassing activity: A suggested mechanism of action. Thrombosis Res, 1981; 21: 181–86.CrossRefGoogle Scholar
  36. 36.
    Lusher JM, Shapiro SS, Palascak JE, Vijaya Rao A, Levine PH, Blatt PM, Haemophilia Study Group: Efficacy of pro-thrombin complex concentrates in haemophiliacs with antibodies to factor VIII: a multicenter therapeutic trial. N Engl J Med, 1980; 303: 421–25.PubMedCrossRefGoogle Scholar
  37. 37.
    Rodeghiero F, Castronovo S, Dini E: Disseminated intravascular coagulation after infusion of FEIBA (Factor VIII Inhibitor Bypassing Activity) in a patient with acquired haemophilia. Thrombosis Haemostasis, 1982; 48: 339–40.Google Scholar
  38. 38.
    Schimpf KL, Zeltsch CH, Zeltsch P: Myocardial infarction complicating activated prothrombin complex concentrate substitution in patient with haemophilia A. Lancet, 1982; ii: 1043.Google Scholar
  39. 39.
    Abildgaard CF: Hazards of Prothrombin-complex concentrates in treatment of haemophilia. N Engl J Med, 1981; 304: 670.PubMedGoogle Scholar
  40. 40.
    Nilsson IM, Hedner U: Characteristics of various factor VIII concentrates used in treatment of haemophilia A. Br J Haematol, 1978; 37: 543.CrossRefGoogle Scholar
  41. 41.
    Bloom AL, Peake IR, Furlong RA, Davies BL: High potency factor VIII concentrate, more effective than cryoprecipitate in a patient with von Willebrand’s disease and inhibitor. Thrombosis Res, 1979; 16: 847.CrossRefGoogle Scholar
  42. 42.
    Jones P: Organisation of a haemophilia service. Haemostasis and Thrombosis, Bloom AL, Thomas DP (eds). Churchill Livingstone 1981; 389–93.Google Scholar
  43. 43.
    Temperley I: National Resources and the Use of factor VIII. In: Bloodtransfusion and problems of bleeding, Smit Sibinga CTh, Das PC, Vanloghem JS (eds). Martinus Nijhoff, 1982; 243–47.Google Scholar
  44. 44.
    Rizza CR, Spooner RJD: Treatment of haemophilia and related disorders in Britain and Northern Ireland during 1976–80: report on behalf of the directors of haemophilia centres in the United Kingdom. Br Med J, 1983; 236: 929–33.CrossRefGoogle Scholar

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© Martinus Nijhoff Publishers. Boston/Dordrecht/Lancaster 1985

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  • C. A. Ludlam

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