The management of acquired haemophilia

David Green*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

23 Scopus citations

Abstract

Acquired haemophilia is a rare but life-threatening bleeding diathesis that usually is caused by autoantibodies to factor VIII (FVIII). The disorder generally affects older persons, often is linked to other autoimmune diseases or cancer, may develop in the postpartum period, and is associated with a distinct bleeding pattern. In contrast to the haemarthroses that typify congenital haemophilia, acquired haemophilia bleeds are characterized by haemorrhage into soft tissue and muscle and the gastrointestinal and genitourinary tracts. A prolonged activated partial thromboplastin time, coupled with a normal prothrombin time, is highly indicative of a circulating anticoagulant; the inhibitor is then quantified with the modified Bethesda assay. Effective control of bleeding is the first step in management and often requires the use of the bypassing agents, either activated prothrombin complex concentrate or recombinant activated factor VII. Elimination of the FVIII antibody is the second goal of therapy. Corticosteroids, cyclophosphamide, and rituximab may eradicate autoantibodies and restore normal haemostasis.

Original languageEnglish (US)
Pages (from-to)32-36
Number of pages5
JournalHaemophilia
Volume12
Issue numberSUPPL. 5
DOIs
StatePublished - Dec 1 2006

Keywords

  • Acquired haemophilia
  • Autoantibodies
  • Bypassing therapy
  • FEIBA
  • Factor VIII
  • Immunosuppressants

ASJC Scopus subject areas

  • Hematology
  • Genetics(clinical)

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