Abstract
INTRODUCTION: Acquired haemophilia A is a rare life- and limb-threatening bleeding disorder if left untreated. Autoimmune thyroiditis is an autoimmune disorder that can be rarely associated with acquired haemophilia. Here we report a case of a 60-year-old woman presenting with cutaneous and muscle haematomas secondary to acquired haemophilia A in association with autoimmune thyroiditis, who was successfully treated with recombinant activated factor VII and immunosuppression. CASE PRESENTATION: A 60-year-old Sri Lankan woman with a background of longstanding hypothyroidism, diabetes mellitus, hypertension, hyperlipidaemia and bronchial asthma developed spontaneous cutaneous purpura and a limb-threatening intramuscular haematoma. Initial coagulation screening revealed prolonged activated partial thromboplastin time of 66.4 seconds (normal range 26 to -36 seconds) and time-dependent inhibitors against factor VIII. She had positive antinuclear antibody and antithyroid peroxidase (microsomal) antibody titre of over 1/80 and 1000IU/mL respectively. The diagnosis was therefore made of acquired haemophilia A in association with autoimmune thyroiditis. Acute limb-threatening bleeding was managed with recombinant activated factor VII (NovoSeven®). Immunosuppressive treatment consisting of oral prednisone 60mg/day and cyclophosphamide 100mg/day was administered in order to remove the factor VIII inhibitor. This treatment led to normalisation of her haemostatic parameters. This case illustrates a very rare association of acquired haemophilia and autoimmune thyroiditis as well as the importance of considering acquired haemophilia as a differential diagnosis of spontaneous bleeding. CONCLUSIONS: Acquired haemophilia should be considered in the differential diagnosis of unexplained bleeding in adults. Treatment of the acute coagulopathy with recombinant activated factor VII and immunosuppressive therapy was successful in this case.