Immunosuppressive therapy (IST) for paroxysmal nocturnal hemoglobinuria (PNH) has been infrequently reported. Four PNH cases were treated with antithymocyte globulin (ATG) at our center. We assessed and reviewed the efficacy and safety of IST for PNH. ATG therapy was performed for progression of cytopenia in 3 classical-type and 1 marrow failure-type PNH cases. ATG was administered at a dose of 15 mg/kg for 5 consecutive days. Hydration and anticoagulant therapy were given as prophylaxis for thrombosis during ATG therapy. Cyclosporine was also given to the 3 classicaltype PNH patients. Three patients showed hemolytic exacerbation and thrombocytopenia during ATG administration, and all needed to receive transfusions of red blood cells and platelets; however, renal failure and thrombosis did not occur. Anemia improved in all cases within 1 year, but thereafter, recurred in 2 cases. ATG therapy is a choice of treatment for PNH, although its mechanism remains unknown.

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A 54-year-old woman had an episode of sudden oral bleeding and generalized petechiae 1 week after a sore throat and diarrhea. On admission, the platelet count was 0.1×10⁴/μl, and the platelet-associated IgG level was elevated. Hyperplasia of megakaryocytes in a bone marrow specimen and aberrant Epstein-Barr virus (EBV) antibody patterns led to a diagnosis of EBV-associated idiopathic thrombocytopenic purpura (ITP). Prednisolone (PSL) promptly restored her platelet count; however, she developed disorientation and affective lability soon after PSL was tapered. Subsequently, she ran a high fever and developed convulsive seizures. T2-weighted MRI demonstrated a high signal area in the subcortical white matter, and no abnormal findings were found on examination of the cerebrospinal fluid. The diagnosis of acute disseminated encephalomyelitis (ADEM) was made and steroid pulse therapy was started, which resulted in remission of the symptoms without recurrence in the following months. This is the first reported case of ADEM following EBV infection during treatment for ITP. Administration of PSL for ITP might mask the presenting clinical picture of ADEM. The possibility of ADEM should be investigated in patients of ITP following viral infection who develop acute encephalopathy.

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We report a 61-year-old man with chronic eosinophilic leukemia (CEL). The patient was referred to our hospital because of pyrexia and eosinophilia. He was diagnosed with CEL based on an increase in blasts and eosinophils in his peripheral blood and bone marrow, and clonal complex karyotypic abnormalities including trisomy 8. The FIP1L1-PDGFRA fusion transcript was not detected by RT-PCR analysis. Although he had no obvious organ damage at diagnosis, pulmonary infiltrates in the right lung and multiple skin nodules over his whole body appeared over 4 months and progressed rapidly, accompanied by a marked increase in blasts in his peripheral blood. CEL with trisomy 8 has been reported to be associated with transformation into acute leukemia and granulocytic sarcoma in the literature. It is notable that the present case was associated with complex karyotypic abnormalities and the exceptionally marked disease progression. Further analyses of clinical data as well as molecular genetic findings of CEL without known karyotypic abnormalities leading to constitutive activation of tyrosine-kinase genes are needed to gain insight into the pathogenesis of CEL and to develop a new disease classification and treatment strategies.

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A 60-year-old man was admitted to our hospital with severe anemia and blood findings showed hemolytic anemia. Further serological examination revealed both warm-reactive autoantibody and cold agglutinin against erythrocytes. The cold agglutinin showed a low titer, 1:32 at 4°C, and had a high thermal amplitude of 30°C or higher, resulting in sufficient activity for hemolysis. Since no underlying disorders could be detected, the diagnosis was idiopathic mixed-type autoimmune hemolytic anemia. Although thrombocytopenia (Evans syndrome) subsequently appeared, corticosteroid was extremely effective for both anemia and thrombocytopenia. In this report we describe a rare case of Evans syndrome associated with mixed-type autoimmune hemolytic anemia, which had a dramatic response to corticosteroid therapy.

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