Acquired haemophilia: a case report and a clinical review

Abstract

BACKGROUND Acquired haemophilia presents later in life and is due to acquired inhibitors to factor VIII. The disorder is very rare (about one case per million population per year) but causes significant morbidity and mortality. CASE REPORT We report the case of a 57 year-old obese man who was admitted to our Day Hospital for anemia. At admission we observed diffuse ecchymoses and a large subcutaneous hematoma in the inferior right hemithorax. Laboratory findings showed severe anemia, a prolonged aPTT with a normal PT and platelet count. The titer of anti-factor VIII inhibitor in Bethesda units resulted very high and confirmed the diagnosis of acquired haemophilia. We used recombinant activated factor VII (rFVIIa) to control active bleeding and prednisone, cyclophosphamide and, lastly, the anti- CD 20 monoclonal antibody (rituximab) to eliminate the factor VIII inhibitors. Unfortunately, on fourth week, the patient suffered an extensive hematoma of the abdominal wall and died. CONCLUSIONS We underline the importance of not overlooking a prolonged aPTT because the bleedings associated with acquired haemophilia can be life-threatening and constitute a medical emergency.