Primary biliary cirrhosis presenting as idiopathic thrombocytopenic purpura with deterioration after splenectomy

A case of primary biliary cirrhosis (PBC) presenting as idiopathic thrombocytopenic purpura is described. The patient, a 59-year-old woman, had, at the time when thrombocytopenia was first observed, an asymptomatic PBC. She responded only temporarily to corticosteroids, and splenectomy had to be performed later. After this operation the platelet count was normalized, but a marked and rapid deterioration of her previously indolent PBC occurred. The time-relationship indicates that the removal of the spleen may be considered the cause of the disease's progression. Possible mechanisms behind this adverse effect of the splenectomy are discussed. After initiation of azathioprine therapy a good response in clinical and laboratory features of the PBC was obtained, although the histological picture has remained unchanged.