Hematological Remission of Primary Myelofibrosis with Antiphospholipid Antibody Following Treatment of Azathioprine

Abstract

A 46-years-old woman was admitted with purpura, nasal bleeding, gum bleeding, gross hematuria, cerebral hemorrhage, and cerebral infarction. The data of her peripheral blood were as follows: WBC 17,500/μl, Hb 5.1 g/dl, PLT 0.3×10⁴/μl, LDH 924 IU/l. Primary myelofibrosis was diagnosed because of bone marrow fibrosis and extramedullary hematopoiesis of the spleen. Furthermore, she revealed marked thrombocytopenia and no response to platelet transfusion, so association of idiopathic thrombocytopenic purpura (ITP) was considered. Though she was treated with high dose predonisolone, high dose γ-globulin, and splenectomy, no hematological improvement was achieved. Administration of azathioprine (100 mg/day) was begun and 2 weeks later, her white blood cell count was approximately 10,000/μl, the platelet count 2.0×10⁴/μl, and no bleeding focus was found. Four weeks later, her hemoglobin content was 13.0 g/dl without blood transfusion. The diagnosis of antiphospholipid syndrome, rather than ITP, was made because of anticardiolipin-β2GPI compex antibody and cerebral infarction. It is interesting that immunosupressant was effective both in primary myelofibrosis and in antiphospholipid syndrome.