Acquired haemophilia A

Česká verzia

Authors: P. Smejkal ^1,2; A. Buliková ^1,2; G. Chlupová ¹; J. Zavřelová ^1,2
Authors place of work: Oddělení klinické hematologie FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Miroslav Penka, CSc. ¹; Lékařská fakulta MU Brno, děkan prof. MUDr. Jiří Mayer, CSc. ²
Published in the journal: Vnitř Lék 2012; 58(7 a 8): 155-162
Category: 60th Birthday prof. MUDr. Miroslav Penka, CSc.

Summary

Acquired haemophilia A is a rare auto-immune disease caused by an inhibitory antibody to factor VIII. Patients with this disorder are at high risk of severe bleeding until the inhibitor has been eradicated. Management of this disorder consists in rapid accurate diagnosis, control of bleeding and eradication of the inhibitor by immunosuppression. The cessation of bleeding is based mainly on recombinant factor VIIa and activated prothrombin complex concentrate which are approximately equally efficacious. Immunosuppression is still based on steroids alone or with combination with cyclopfosphamide which may result in a higher rate of remission. New drugs as rituximab or cyclosporine A are the second line option. In case of life-threatening bleeding immunoadsorption and high dose of factor VIII could be advantageous.

Key words:
acquired factor VIII inhibitor – acquired haemophilia A – immunosuppression – bleeding – treatment

Zdroje

1. Collins PW, Percy CL. Advances in the understanding of acquired haemophilia A: implication for clinical practice. Br J Haematol 2009; 148: 183–194.

2. Oldenburg J, Zeitler H, Pavlova A. Genetic markers in acquired haemophilia. Haemophilia 2010; 16 (Suppl 3): 41–45.

3. Knoebl P, Marco P, Baudo F et al. EACH2 Registry Contributors. Demografic and clinical data in acquired hemophilia A: results from the European Haemophilia Registry (EACH2). J Thromb Haemost 2012; 10: 622–631.

4. Green D, Lechner K. A Survey of 215 Non-Hemophilic Patients with Inhibitors to Factor VIII. Thromb Haemost 1981; 45: 200–203.

5. Hay CR. Acquired haemophilia. Baillieres Clin Haematol 1998; 11: 287–303.

6. Sood SL, Kessler CM. Acquired inhibitors to factor VIII. In: Lee CA, Berntorp EE, Hoots WK (eds). Textbook of Hemophilia. Oxford: Blackwell Publishing 2010: 81–87.

7. Delgado J, Jimenez-Yuste V, Hernandez-Navarro F et al. Acquired haemophilia: Review and meta-analysis focused on therapy and prognostic factors. Br J Haematol 2003; 121: 21–35.

8. Morrison AE, Ludlam CA, Kessler C. Use of porcine factor VIII in the treatment of patients with acquired hemophilia. Blood 1993; 81: 1513–1520.

9. Collins PW, Hirsch S, Baglin TP et al. UK Haemophilia Centre Doctors’ Organisation. Acquired hemophilia A in the United Kingdom: a 2-year national surveillance study by the United Kingdom Heamophilia Centre Doctors’ Organisation. Blood 2007; 109: 1870–1877.

10. Hay CR, Brown S, Collins PW et al. The diagnosis and management of factor VIII and IX inhibitors: a guideline from the United Kingdom Haemophilia Centre Doctors Organisation. Br J Haematol 2006; 133: 591–605.

11. Feinstein DI, Green D, Federici AB et al. Diagnosis and Management of Patients with Spontaneously Acquired Inhibitors of Coagulation. American Society of Hematology Education Programm Book Hematology 1999: 192–208.

12. Goldsmith JC. Diagnosis of Factor VIII versus Nonspesific Inhibitors. Semin Hematol 1993; 30 (Suppl 1): 3–6.

13. Cohen AJ, Kessler CM. Acquired inhibitors. Baillieres Clin Hematol 1996; 9: 331–354.

14. Buliková A, Zavřelová J, Penka M. Antifosfolipidový syndrom v roce 2009. Vnitř Lék 2009; 55: 253–262.

15. Hay CR, Negrier C, Ludlam CA. The Treatment of Bleeding in Acquired Haemophilia with Recombinant Factor VIIa: A Multicentre Study. Thromb Haemost 1997; 79: 1463–1467.

16. Holme PA, Brosstad F, Tjønnfjord GE. Acquired haemophilia: management of bleeds and immune therapy to eradicate autoantibodies. Haemophilia 2005; 11: 510–515.

17. Nemes L, Tengborn L, Collins PW et al. Acquired Haemophilia A and Pregnancy/Postpartum – a Report From a European Registry. Blood 2010; 116: Abstract 717.

18. Sallah S, Wan JY. Inhibitors against factor VIII in patients with cancer. Analysis of 41 patients. Cancer 2001; 9: 1067–1074.

19. Michiels JJ. Acquired Hemophilia A in Women Postpartum: Clinical Manifestations, Diagnosis, and Treatment. Clin Appl Thromb Hemost 2000; 6: 82–86.

20. Hauser I, Schneider B, Lechner K. Post-partum factor VIII inhibitors. A review of the literature with special reference to the value of steroid and immunosuppressive treatment. Thromb Haemost 1995; 73: 1–5.

21. Collins PW. Management of acquired haemophilia A – more questions than answers. Blood Coag Fibrionol 2003; 14 (Suppl 1): 23–27.

22. Collins PW. Acquired haemophilia A: immunine suppression. Haematologica 2004; 89: (Suppl 5): 3–7.

23. Solymoss S. Postpartum acquired factor VIII inhibitors: results of a survey. Am J Hematol 1998; 59: 1–4.

24. Baudo F, de Cataldo F. Italian Association of Haemophilia Centres (AICE): Register of acquired factor VIII inhibitors (RIIA). Acquired factor VIII inhibitors in pregnancy: data from the Italian Haemophila Register relevant to clinical practice. BJOG 2003; 110: 311–314.

25. Collins PW. Management of acquired haemophilia A. J Thromb Haemost 2011; 9 (Suppl 1): 226–235.

26. Knoebl P, Baudo F, Collins PW et al. EACH2 Registry Contributors. Management of Bleeding In Acquired Hemophilia: Results of the European Acquired Hemophilia Registry (EACH2). Blood 2010; 116: Abstract 716.

27. Jansen M, Schmaldienst S, Banyai S et al. Treatment of coagulation inhibitors with extracorporeal immunoadsorption (Ig-Theasorb). Br J Haematol 2001; 112: 91–97.

28. Zeitler H, Ulrich-Merzenich G, Goldmann G et al. The prevalence of the bleeding severity in the treatment of acquired hemophilia – an update of a single-centre experience with 67 patients. Haemophilia 2010; 16: 95–101.

29. Söhngen D, Specker C, Bach D et al. Acquired factor VIII inhibitors in nonhemophilic patiens. Ann Hematol 1997; 74: 89–93.

30. Grünewald M, Beneke H, Güthner C et al. Acquired haemophilia: experience with a standardized approach. Haemophilia 2001; 7: 164–169.

31. Ji L, Yang R, Yang D et al. Prednisone and low-dose activated prothrombin complex concentrates for FVIII inhibitor in nonhaemophilic patients. Haemophilia 1998; 4: 721–724.

32. Sallah S. Treatment of acquired haemophilia with factor eight inhibitor bypassing activity. Haemophilia 2004; 10: 169–173.

33. Baudo F, de Cataldo F, Gaidano G. Italian registry of acquired hemophilia. Treatment of acquired factor VIII inhibitor with recombinant activated factor VIIa: data from the Italian registry of acquired hemophilia. Haematologica 2004; 89: 759–761.

34. Sumner MJ, Geldziler BD, Pedersen M et al. Treatment of acquired haemophilia with recombinant activated FVII: a critical appraisal. Haemophilia 2007; 13: 451–461.

35. Sood SL, Konkle BA, Kessler CM et al. Treatment of Acute Bleeds in acquired Hemophilia: An Updated Analysis From the Hemophilia and Thrombosis Research Society (HTRS) Registry. Blood 2009; 115: Abstract 3499.

36. Green D, Rademaker AW, Briët E. A prospective, randomised trial of prednisone and cyclophosphamide in the treatment of patients with factor VIII autoantibodies. Thromb Haemost 1993; 70: 753–757.

37. Collins PW, Baudo F, Knoebl P et al. Inhibitor Eradication In Acquired Haemophilia A: Final Results of European Acquired Haemophilia Registry (EACH2). Blood 2010; 116: Abstract 715.

38. Boggio LN, Green D. Acquired hemophilia. Rev Clin Exp Hematol 2001; 5: 389–404.

39. Lian EC, Larcada AF, Chiu AY. Combination immunosuppressive therapy after factor VIII infusion for acquired factor VIII inhibitor. Ann Intern Med 1998; 110: 774–778.

40. Lian EC, Villar MJ, Noy LI et al. Acquired factor VIII inhibitor treated with cyclophosphamide, vincristine, and prednisone. Am J Hematol 2002; 69: 294–295.

41. Nemes L, Pitlik E. New protocol for immune tolerance induction in acquired hemophilia. Haematologica 2000; 85 (Suppl 10): 64–68.

42. Nemes L. The use of immune tolerance induction regimes for acquired hemophilia. Haemophilia 2004; 10 (Suppl 3): 54.

43. Schwarz RS, Gabriel DA, Aledort LM et al. A prospective study of treatment of acquired (autoimmune) factor VIII inhibitors with high-dose intravenous gammaglobulin. Blood 1995; 86: 797–804.

44. Dykes AC, Walker ID, Lowe GD et al. Combined prednisolone and intravenous immunoglobulin treatment for acquired factor VIII inhibitors: a 2-year review. Haemophilia 2001; 7: 160–163.

45. Huth-Kühne A, Baudo F, Collins P et al. International recommendations on the diagnosis and treatment of patients with acquired hmophilia A. Haematologica 2009; 94: 566–575.

46. Hart HC, Kraaijenhagen RJ, Kerckhaert LA et al. A patient with spontaneous factor VIII:C autoantibody: successful treatment with cyclosporine. Transplant Proc 1988; 20 (Suppl 4): 323–328.

47. Au WY, Lam CC, Kong YL. Successful treatment of acquired factor VIII inhibitor with cyclosporine. Haemophilia 2004; 10: 98–100.

48. Wano Y, Kang Y, Masaki H et al. Cyclosporine A as an effective treatment for a patient with acquired hemophilia A complicated with diabetes mellitus and ischemic heart disease. Japan J Clin Hematol 2005; 46: 1100–1104.

49. Leinøe EB, Jørgensen M, Nielsen JD. Acquired factor VIII deficiency treated with cyclosporine. Ugeskr Laeger 1999; 161: 4762–4763.

50. Mikami T, Masauzi N, Niwa J et al. Acquired factor VIII inhibitors in patient associated with neurosurgery: case report and review of the literature. Neurosurgery 2005; 57: 595.

51. Stasi R, Bruneti M, Stipa E et al. Selective B-cell depletion with rituximab for the treatment of patients with acquired hemophilia. Blood 2004; 103: 4424–4428.

52. Aggarwal A, Grewal R, Green RJ et al. Rituximab for autoimmune haemophilia: a proposed treatment algorithm. Haemophilia 2005; 11: 13–19.

53. Franchini M. Rituximab in the treatment of adult acquired hemophilia A: A systematic review. Crit Rev Oncol Hematol 2007; 63: 47–52.

54. Mazzucconi MG, Biondo F, Santoro C. Rituximab in the Treatment of Postpartum Acquired Hemophilia A. Journal of coagulation disorders 2009; 1: 47–54.

55. Penka M, Blatný J, Matýšková M et al. Hemofilie z pohledu zajištění péče o nemocné. Vnitř Lék 2009; 55 (Suppl 1): 48–54.

56. Český národní hemofilický program. http://cnhp.registry.cz.

57. http://www.novoseven.cz/proHCP/ziskhem.html.