Hairy cell leukemia in association with thrombotic thrombocytopenic purpura and factor VIII antibodies

A unique patient is reported with longstanding hairy cell leukemia who manifested two distinct abnormalities of factor VIII; factor VIII antibodies and recurrent thrombotic thrombocytopenic purpura (TTP). The patient presented in 1977 with splenomegaly and pancytopenia and was diagnosed with hairy cell leukemia and was treated with splenectomy. In 1989 he received interferon-alpha because of a relapse which resulted in a hematologic remission. Hospitalization on two occasions for gross hematuria was caused by the development of a factor VIII antibody. He was successfully treated on both occasions with cyclophosphamide, prednisone and active prothrombin complex (FEIBA). In October 1991 he presented with microangiopathic hemolytic anemia and thrombocytopenia. A diagnosis of thrombotic thrombocytopenic purpura (TTP) was made. Repeat bone marrow biopsy showed hairy cell leukemia. The patient responded to treatment with plasmapheresis, fresh frozen plasma replacement and prednisone. He had two subsequent relapses with the last being refractory and subsequently fatal. During the initial manifestation of TTP and in follow-up evaluation unusually large von Willebrand factor multimers were demonstrated.