The effect and safety of combination prophylaxis for hepatic veno-occlusive disease (VOD) following stem cell transplantation (SCT) was retrospectively evaluated in a total of 53 children who survived until day 30. Prophylaxis was started on the day before conditioning, and heparin (10 unit/kg/hr) alone was used in 6 patients, PGE1 (5 ng/kg/min) plus heparin plus PTX (200 mg/day) in 17 patients, lipo-PGE1 (0.5 ng/kg/min) plus heparin plus PTX in 7 patients, and lipo-PGE1 plus heparin in 23 patients. Diagnosis of VOD was made based on McDonald's criteria in 5 cases (9.4%), but not on Jones' criteria. No statistically significant difference was observed in the incidence of VOD among each prophylaxis group. The degree of VOD was mild in all of 5 cases, and all recovered with continuation of the prophylactic procedure. Each prophylactic procedure was performed without any significant adverse effect except for seizure induced by lipo-PGE1 in one patient with Lennox syndrome. Since both the incidence and fatality rate of VOD in children undergoing SCT are approximately 20% according to most of the previous reports, the present study suggests the effectiveness of combination prophylaxis.

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A 72-year-old woman presented with a progressive swollen and painful left knee over a period of a month. The patient had a history of tuberculous arthritis of the left knee joint at the age of 1-2 years. Since then the knee showed a deformity and contraction. MRI scan demonstrated a soft tissue tumor measuring 7×8×6 cm in the front of the knee, a biopsy specimen taken from which revealed large B-cell lymphoma. The tumor cells showed a phenotype of CD3- CD5- CD10- CD20+ CD21- BCL2+, and surface immunoglobulin M-κ. Since the lymphoma cells were found to be negative for Epstein-Barr virus encoded RNA by means of in situ hybridization, the DLBCL in this patient was thought to be caused by a different mechanism from pyothorax-associated lymphoma. The patient was treated with chemotherapy (CHOP) followed by radiotherapy, and obtained partial remission. There is no evidence of progression seven months after diagnosis.

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A 50-year-old man was admitted to our hospital because of thrombocytopenia during a follow up study of diffuse large B-cell lymphoma in second complete remission. He was diagnosed as having therapy-related acute myelgenous leukemia (t-AML) on the basis of the bone marrow findings and his chemotherapeutic agent history including alkylating agents. Complete remission was achieved by induction chemotherapy. Although allogeneic stem cell transplantation was thought to be needed, the patient was thought to be ineligible for any myeloablative conditioning regimen because of his age and the history of long term chemoradiotherapy. A non-myeloablative regimen was thus selected. After preconditioning with fludarabine, cyclophosphamide and cytarabine, the patient underwent peripheral blood stem cell transplantation from an HLA identical sibling donor. Complete donor chimeras were obtained on day 28 after transplantation. Regimen related toxicities over grade 2 were not observed. Although he suffered from mild chronic graft-versus-host disease (GVHD), he is in good condition without any signs of relapse during a follow-up period of 33 months. It is suggested that non-myeloablative transplantation is feasible and benefical for patients with t-AML who are often ineligible for myeloablative transplants because of their histories of long term chemoradiotherapies.

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This case report describes a hairy B cell lymphoproliferative disorder (HBLD) with clinical and hematological features resembling hairy cell leukemia. The patient was a 29-year-old female who demonstrated atypical lymphocytes in her peripheral blood. Physical examination demonstrated splenomegaly, but there were no palpable superficial lymph nodes. Hematological examination showed a leukocyte count of 10.6×10³/mm³ with 41% atypical lymphocytes. Bone marrow examination showed a normal cellular and an atypical lymphocyte count of 42%. The atypical lymphocytes had microvilli and prominent membranous ruffles on their surfaces. Atypical lymphocytes expressed CD5-CD10-CD11c+CD19+CD20+CD23-CD25- on the surface of the cells on examination by with a fluorescence activated cell sorter. Although these findings were similar to hairy cell leukemia, Japanese variant, the surface marker of the κ chain and λ chain was unbiased and studies of immunoglobulin gene rearrangements and expression showed polyclonal proliferation of B cells. Therefore, we diagnosed this patient as having HBLD. Because she did not demonstrate anemia or thrombocytopenia, she is not currently receiving medication. To date, the atypical lymphocyte count has not changed.

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A 17-year-old female was admitted to our hospital because of pyrexia and thrombocytopenia in May 2002. Laboratory examination showed a platelet count of 50,000/μl with an increased level of fibrinogen degradation product, leading to a diagnosis of disseminated intravascular coagulation (DIC). Gabexate mesilate was intravenously administrated without any effects. Several days later, erythema, joint pain and neck lymphadenopathy developed sequentially. The patient was diagnosed as having adult-onset Still disease (AOSD) complicated with DIC. Moreover, serum inflammatory cytokine levels had increased and activated macrophages were observed in the bone marrow, suggesting the presence of macrophage activation syndrome. After additional treatments with dalteparin and aspirin, the clinical symptoms and laboratory findings associated with AOSD and DIC disappeared. Although this was a severe case of AOSD associated with preceding DIC, the AOSD symptoms resolved in this patient with the treatment of the DIC and with aspirin only without any relapse.

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A 39-year-old woman received an allogeneic peripheral blood stem cell transplantation from her daughter for secondary myelodysplastic syndrome. On day +12, a cough and fever developed. Chest X-ray and computed tomography demonstrated a consolidation in the left lung. A diagnosis of pulmonary zygomycosis was made on the histology from a transbronchial lung biopsy. Although amphotericin B (AMPH-B) showed efficacy, dose reduction was necessary because of renal toxicity. The patient died of pulmonary zygomycosis, confirmed by an autopsy. We strongly hope for authorization as soon as possible of the use of such a drug as liposomal AMPH-B which appears less toxic.

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