A case of idiopathic acquired haemophilia - A with massive subcutaneous bleeding

Abstract

We report a case of a 69-year-old male, a known epileptic on phenytoin for 40 years, presented with ecchymotic patches over the right upper and lower extremities. There was a history of right leg pain 3 weeks before presentation, when he was misdiagnosed as deep venous thrombosis (DVT) on the basis of venous Doppler at a nursing home. He was treated with low-molecular-weight heparin followed by acenocoumarol. Subsequently, he developed subcutaneous bleeding followed by sudden drop in haemoglobin, and anticoagulant measures were withheld. He continued to have fresh crops of ecchymosis even 10 days after withdrawal of anticoagulants; hence, he was shifted to our hospital. On evaluation, his activated partial thromboplastic time (APTT) was found elevated and anaemia was present, while prothrombin time and thrombin time were normal. His factor VIII level was identified as low (1%) and factor VIII inhibitor level was elevated. With diagnosis of acquired haemophilia A, he was treated with recombinant factor VII, steroid and cyclophosphamide. There was no cause identified for acquired haemophilia A, even after extensive work-up for malignancy, viral diseases, connective disorders and coagulating disorders. Hence, the diagnosis of idiopathic acquired haemophilia was considered.

Keywords

• acquired haemophilia-a
• phenytoin
• recombinant factor vii