Ervervet hemofili: diagnostikk og behandling av 38 pasienter

Abstract

Acquired hemophilia is a rare, but potentially life threatening bleeding disorder. The disease is caused by autoantibodies against coagulation factors, most often FVIII. The disease is characterized by spontaneous bleeding in patients with no previous personal or family history of bleedings. This is a follow-up study of Holme et al.[1] from 2005 and includes patients diagnosed with acquired hemophilia at Oslo University Hospital, Rikshospitalet between 1997 and 2011. A total of 38 patients with a median age of 80,5 years were identified, 55 % were men. Bleeding was the cause of death in 5,3 % of the patients in this study. Diffuse bleeds was the most common presentation of acquired hemophilia and occurred in 39,4 % of the patients. 47,4 % of the patients had comorbidity connected to cancer or autoimmune disease. Bleeds treated with activated prothrombin complex concentrate (aPCC) and recombinant factor VIIa (rFVIIa) were evaluated. APCC was used as prophylactic treatment in 4 surgeries and 11 bleeds were treated with aPCC. The treatment with aPCC had good or excellent effect in 93,3 % of the 15 episodes. 2 bleeding episodes were treated with rFVIIa with excellent effect in 50 % of the bleeds. 25 patients received eradication therapy with steroids and cyclophosphamide, of which follow-up data was known in 21 patients. 95,2 % of the patients with follow-up data received a beneficial response to the inhibitor and APTT. A complete response within 6 months was seen in 57,1 % of patients treated with the combination of steroids and cyclophosphamide.