Management of Acquired Hemophilia: A Literature Review

Review Article

Management of Acquired Hemophilia: A Literature Review

Pål Andrè Holme ¹ and Geir Erland Tjønnfjord ^{1 2}
Affiliations: Department of Haematology, Oslo University Hospital, Rikshospitalet;  ¹ Faculty Division Rikshospitalet ² , University of Oslo, Norway


ABSTRACT

Acquired hemophilia is a rare, but often severe, bleeding disorder caused by autoantibodies against a coagulation factor, usually factor VIII (FVIII). The most common clinical presentations are diffuse bruising, soft tissue bleeds, and hematuria. As acquired hemophilia is associated with severe bleeding complications with high mortality, early diagnosis and intervention are crucial. The bypassing agents, recombinant activated factor VII (rFVIIa) and activated prothrombin complex concentrate (aPCC), have been shown to be effective as first‐line treatment for bleeds, and treatment should be switched to the other product if the first choice fails. Starting immune therapy to eradicate the autoantibodies should not be postponed. The combination of oral corticosteroids and cyclophosphamide is effective in eradicating the neutralizing autoantibodies, and should probably be the choice for first‐line treatment. Rituximab is a promising drug in acquired hemophilia, but should perhaps be limited to second‐line treatment.

This paper reviews the current literature in management of bleeds and immune therapy to eradicate autoantibodies in patients with acquired hemophilia.

Keywords: acquired hemophilia, FVIII inhibitor, FIX inhibitor, activated prothrombin complex concentrates, rFVIIa, immunosuppression
Correspondence: Pål Andrè Holme, Department of Haematology, Oslo University Hospital, Rikshospitalet, Oslo, Norway. Tel: (47)‐23070000; Fax: (47)‐23070470; e‐mail: holme@rikshospitalet.no