open access

Vol 13, No 3 (2020)
Review paper
Published online: 2020-12-01
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A practical approach to the patient with acquired haemophilia A

Magdalena Górska-Kosicka, Michał Wójciak, Jerzy Windyga
Journal of Transfusion Medicine 2020;13(3):176-185.

open access

Vol 13, No 3 (2020)
REVIEW ARTICLE
Published online: 2020-12-01

Abstract

Acquired haemophilia (AHA) is severe bleedinig disorder caused by autoantibodies against coagulation factor VIII (FVIII).  The antibodies inhibit the coagulation function of FVIII and lead to a decrease in its activity in the patient's plasma. AHA occurs mainly in the elderly and more than half of the cases are idiopathic. The conditions associated with its occurrence include autoimmune diseases, neoplasms and up to 12 months after delivery. The clinical picture is dominated by extensive ecchymosis, less often intramuscular and retroperitoneal hematomas or central nervous system hemorrhages. In laboratory studies, AHA is characterized by isolated activated partial thromboplastin time (APTT) prolongation, decreased FVIII activity and the presence of a FVIII inhibitor. Although patients with AHA usually have severe hemorrhagic diathesis, asymptomatic forms may also occur, with the accidentally detected APTT prolongation as the only abnormality. The aims of AHA treatment are to control the bleeding and to eliminate the inhibitor.

Abstract

Acquired haemophilia (AHA) is severe bleedinig disorder caused by autoantibodies against coagulation factor VIII (FVIII).  The antibodies inhibit the coagulation function of FVIII and lead to a decrease in its activity in the patient's plasma. AHA occurs mainly in the elderly and more than half of the cases are idiopathic. The conditions associated with its occurrence include autoimmune diseases, neoplasms and up to 12 months after delivery. The clinical picture is dominated by extensive ecchymosis, less often intramuscular and retroperitoneal hematomas or central nervous system hemorrhages. In laboratory studies, AHA is characterized by isolated activated partial thromboplastin time (APTT) prolongation, decreased FVIII activity and the presence of a FVIII inhibitor. Although patients with AHA usually have severe hemorrhagic diathesis, asymptomatic forms may also occur, with the accidentally detected APTT prolongation as the only abnormality. The aims of AHA treatment are to control the bleeding and to eliminate the inhibitor.
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Keywords

acquired haemophilia A; factor VIII; bypassing agents; inhibitor, bleeding

About this article
Title

A practical approach to the patient with acquired haemophilia A

Journal

Journal of Transfusion Medicine

Issue

Vol 13, No 3 (2020)

Article type

Review paper

Pages

176-185

Published online

2020-12-01

Bibliographic record

Journal of Transfusion Medicine 2020;13(3):176-185.

Keywords

acquired haemophilia A
factor VIII
bypassing agents
inhibitor
bleeding

Authors

Magdalena Górska-Kosicka
Michał Wójciak
Jerzy Windyga

References (40)
  1. Windyga J, Chojnowski K, Klukowska A, et al. W imieniu Grupy Roboczej ds. Hemostazy Polskiego Towarzystwa Hematologów i Transfuzjologów: Polskie zalecenia postępowania w nabytej hemofilii A. Med. Prakt. 2011; 10: 42–51.
  2. Windyga J, Baran B, Odnoczko E, et al. Wytyczne postępowania w nabytej hemofilii A. Ginekologia i Perinatologia Praktyczna. 2018, tom 3, nr. ; 4: 175–188.
  3. Tiede A, Collins P, Knoebl P, et al. International recommendations on the diagnosis and treatment of acquired hemophilia A. Haematologica. 2020; 105(7): 1791–1801.
  4. Knoebl P, Marco P, Baudo F, et al. EACH2 Registry Contributors. Demographic and clinical data in acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2). J Thromb Haemost. 2012; 10(4): 622–631.
  5. 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 Haemophilia Centre Doctors' Organisation. Blood. 2007; 109(5): 1870–1877.
  6. Baudo F, Mostarda G, de Cataldo F. Acquired factor VIII inhibitors in pregnancy: data from the Italian Haemophilia Register relevant to clinical practice. BJOG: An International Journal of Obstetrics and Gynaecology. 2003; 110(3): 311–314.
  7. Franchini M, Zaffanello M, Lippi G. Acquired hemophilia in pediatrics: a systematic review. Pediatr Blood Cancer. 2010; 55(4): 606–611.
  8. Windyga J, Zdziarska J, Chojnowski K, et al. Acquired hemophilia A in Poland a report from AHA-POL registry. J Thromb Haemost 2015;13;2. ; 758: PO011–WED.
  9. Kruse-Jarres R, Kempton CL, Baudo F, et al. Acquired hemophilia A: Updated review of evidence and treatment guidance. Am J Hematol. 2017; 92(7): 695–705.
  10. Toschi V, Baudo F. Diagnosis, laboratory aspects and management of acquired hemophilia A. Intern Emerg Med. 2010; 5(4): 325–333.
  11. Windyga J, Chojnowski K, Klukowska A, et al. Część II: Wytyczne postępowania w hemofilii A i B powikłanej inhibitorem czynnika VIII i IX (2 wydanie). Acta Haematologica Polonica. 2017; 48(3): 137–159.
  12. Huth-Kühne A, Baudo F, Collins P, et al. International recommendations on the diagnosis and treatment of patients with acquired hemophilia A. Haematologica. 2009; 94(4): 566–575.
  13. Baudo F, Collins P, Huth-Kuhne A, et al. Management of bleeding in acquired hemophilia A: results from the European Acquired Haemophilia (EACH2) registry. Blood. 2012;120:39–46. .
  14. Sallah S. Treatment of acquired haemophilia with factor eight inhibitor bypassing activity. Haemophilia. 2004; 10(2): 169–173.
  15. Borg JY, Négrier C, Durieu I, et al. FEIBHAC Study Group. FEIBA in the treatment of acquired haemophilia A: results from the prospective multicentre French 'FEIBA dans l'hémophilie A acquise' (FEIBHAC) registry. Haemophilia. 2015; 21(3): 330–337.
  16. Tiede A, Worster A. Lessons from a systematic literature review of the effectiveness of recombinant factor VIIa in acquired haemophilia. Ann Hematol. 2018; 97(10): 1889–1901.
  17. Ma AD, Kessler CM, Al-Mondhiry HAB, et al. US experience with recombinant factor VIIa for surgery and other invasive procedures in acquired haemophilia: analysis from the Hemostasis and Thrombosis Research Society Registry. Haemophilia. 2016; 22(1): e18–e24.
  18. Knöbl P. Prevention and Management of Bleeding Episodes in Patients with Acquired Hemophilia A. Drugs. 2018; 78(18): 1861–1872.
  19. Árokszállási A, Rázsó K, Ilonczai P, et al. A decade-long clinical experience on the prophylactic use of activated prothrombin complex concentrate in acquired haemophilia A: a case series from a tertiary care centre. Blood Coagul Fibrinolysis. 2018; 29(3): 282–287.
  20. Kessler CM, Knöbl P. Acquired haemophilia: an overview for clinical practice. Eur J Haematol. 2015; 95 Suppl 81: 36–44.
  21. Kempton CL, Abshire TC, Deveras RA, et al. Pharmacokinetics and safety of Obi-1, arecombinant B domain-deleted porcine factor VIII, in subjects with haemophilia A. Haemophilia. 2012; 18: 798–804. .
  22. Kruse-Jarres R, St-Louis J, Greist A, et al. Efficacy and safety of OBI-1, an antihaemophilic factor VIII (recombinant), porcine sequence, in subjects with acquired haemophilia A. Haemophilia. 2015; 21(2): 162–170.
  23. Tarantino MD, Cuker A, Hardesty B, et al. Recombinant porcine sequence factor VIII(rpFVIII) for acquired haemophilia A: practical clinical experience of its use in seven patients. Haemophilia 2017; 23: 25–32. .
  24. Martin K, Kasthuri R, Mooberry MJ, et al. Lower doses of recombinant porcine factor VIII maintain excellent haemostatic efficacy. Haemophilia. 2016; 22(6): e549–e551.
  25. Charakterystyka Produktu Leczniczego Obizur: https://www.ema.europa.eu/en/documents/product-information/obizur-epar-product-information_pl.pdf.
  26. Burness CB, Scott LJ. Susoctocog Alfa: A Review in Acquired Haemophilia A. Drugs. 2016; 76(7): 815–821.
  27. Fosbury E, Drebes A, Riddell A, et al. Review of recombinant anti-haemophilic porcine sequence factor VIII in adults with acquired haemophilia A. Ther Adv Hematol. 2017; 8(9): 263–272.
  28. Windyga J, Stefanska-Windyga E, Odnoczko E, et al. Activated prothrombin complex concentrate in combination with tranexamic acid: a single centre experience for the treatment of mucosal bleeding and dental extraction in haemophilia patients with inhibitors. Haemophilia. 2016; 22(5): e465–e468.
  29. Pasca S, Ambaglio C, Rocino A, et al. FAIR Study Group. Combined use of antifibrinolytics and activated prothrombin complex concentrate (aPCC) is not related to thromboembolic events in patients with acquired haemophilia A: data from FAIR Registry. J Thromb Thrombolysis. 2019; 47(1): 129–133.
  30. Holmström M, Tran HTT, Holme PA. Combined treatment with APCC (FEIBA®) and tranexamic acid in patients with haemophilia A with inhibitors and in patients with acquired haemophilia A--a two-centre experience. Haemophilia. 2012; 18(4): 544–549.
  31. Holstein K, Liu X, Smith A, et al. Bleeding and response to hemostatic therapy in acquired hemophilia A: results from the GTH-AH 01/2010 study. Blood. 2020; 136(3): 279–287.
  32. Franchini M, Lippi G. Acquired factor VIII inhibitors. Blood. 2008; 112(2): 250–255.
  33. Franchini M, Vaglio S, Marano G, et al. Acquired hemophilia A: a review of recent data and new therapeutic options. Hematology. 2017; 22(9): 514–520.
  34. Collins P, Baudo F, Knoebl P, et al. EACH2 registry collaborators. Immunosuppression for acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2). Blood. 2012; 120(1): 47–55.
  35. Tiede A, Klamroth R, Scharf RE, et al. Prognostic factors for remission of and survival in acquired hemophilia A (AHA): results from the GTH-AH 01/2010 study. Blood. 2015; 125(7): 1091–1097.
  36. Vautier M, de Boysson H, Creveuil C, et al. Influence of factor VIII level and its inhibitor titer on the therapeutic response to corticosteroids alone in the management of acquired hemophilia: A retrospective single-center study. Medicine (Baltimore). 2016; 95(48): e5232.
  37. Charlebois J, Rivard GÉ, St-Louis J. Management of acquired hemophilia A: Review of current evidence. Transfus Apher Sci. 2018; 57(6): 717–720.
  38. Nemes L, Pitlik E. New protocol for immune tolerance induction in acquired hemophilia. Haematologica. 2000; 85(10 Suppl): 64–68.
  39. Zeitler H, Ulrich-Merzenich G, Hess L, et al. Treatment of acquired hemophilia by the Bonn-Malmo Protocol: documentation of an in vivo immunomodulating concept. Blood. 2005; 105(6): 2287–2293.
  40. Borg JY, Guillet B, Le Cam-Duchez V, et al. SACHA Study Group. Outcome of acquired haemophilia in France: the prospective SACHA (Surveillance des Auto antiCorps au cours de l'Hémophilie Acquise) registry. Haemophilia. 2013; 19(4): 564–570.

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