Acquired haemophilia in association with organ-specific autoimmune disease

A 72-year-old woman presented with multiple sites of soft tissue bleeding and a left ulnar neuropathy due to a compartment syndrome. There were also clinical signs of hypothyroidism. Factor VIII:C (FVIII) levels were 6% and a FVIII inhibitor with a titre of 9 Bethesda units was detected. She had biochemical evidence of hypothyroidism and high titres of antithyroid antibodies were detected. Two episodes of life- or limb-threatening haemorrhage were successfully treated with FEIBA. The inhibitor disappeared following 2 months of immunosuppression with oral cyclophosphamide and corticosteroids. She then developed autoimmune haemolytic anaemia, which responded to further immunosuppression with oral prednisolone. The association between acquired haemophilia and systemic autoimmune disorders such as systemic lupus erythematosis is well recognized, but our description of an association with organ-specific autoimmunity is more unusual.