MANAGEMENT OF BLEEDING IN A PATIENT WITH ACQUIRED HAEMOPHILIA A: A CASE REPORT

Acquired haemophilia A (AHA) is a blood clotting disorder caused by the presence of autoantibodies that interfere with the function of factor VIII, often called factor VIII inhibitor (1). It has a prevalence of about one per million per year. Acquired haemophilia A is usually associated with autoimmune diseases, malignancies, skin disorders, drug interactions, and the postpartum period, and as many as 50% of cases have unknown causes (2). Bleeding often occurs under the skin (purpura/ecchymosis) and in soft tissues, and rarely in the joints (haemarthroses) (3). The presence of Factor VIII inhibitors causes a disruption in the intrinsic coagulation pathway, leading to prolongation of the activated partial thromboplastin time (APTT) parameter, which does not improve after mixing test, and has low Factor VIII activity (2). The management of patients with AHA aims to control bleeding and its complications and to eradicate factor VIII inhibitors. Immunosuppressive agents are usually used to eradicate factor inhibitors. In this study, we report a case of acquired haemophilia A presenting with spontaneous unprovoked bruising and discuss the approach to diagnosis and how to alert the clinician to suspect this potentially rare but devastating disease. A 67-year-old man presented to the emergency department with bruising on his left hand and right leg for approximately 10 days. The patient had no history of trauma. He had a history of hypertension and chronic obstructive pulmonary disease. Approximately 2 months prior to presentation, he had complained of bruising on his arms after taking medication (etodolac, ampicillin-sulbactam) (Figure 1). There had been no bleeding after the cholecystectomy. Physical examination revealed an anteroposterior diffuse haematoma extending from the right inguinal to the popliteal area and a haematoma on the dorsal aspect of the left hand (Figure 2). Other systemic examinations were normal. All laboratory results showed the initial complete blood count; normochromic anaemia, normal platelet count and prolonged activated partial thromboplastin time (APTT) with normal prothrombin time (PT). Liver and renal function tests were within normal limits. The test results showed very low factor VIII activity with normal von Willlebrand factor and fibrinogen levels (factor VIII activity: