Although the environment serves as a reservoir for a variety of microorganisms, it is rarely implicated in disease transmission. However, exposure to environmental opportunistic pathogens (e.g., ^Aspergillus spp. and ^Legionella spp.) or airborne pathogens (e.g., ^{Mycobacterium tuberculosis} and varicella-zoster virus) may result in infections with significant morbidity and/or mortality in hematopoietic stem cell transplantations (HSCTs). In HSCTs, cyclosporine or tacrolimus (FK506) is administered in combination with other immunosuppressive agents (e.g., methotrexate or corticosteroids) to prevent and treat graft vs host disease (GVHD). Although cyclosporine is effective in preventing GVHD, its use entails greater hazards for infectious complications. Although survival rates for certain autologous recipients have improved, infection remains a leading cause of death among allogeneic transplants and is a major cause of morbidity among autologous HSCTs. Therefore, adherence to established standards (e.g. hand washing, proper ventilation for specialized care areas, and proper use of disinfectants) should be taken and can result in better patient outcomes in HSCTs. In this article, infection control strategies have been addresssed, especially for the environment.

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Kasabach-Merritt syndrome is characterized by thrombocytopenia, consumption coagulopathy associated with massive hemangiomas. The pathogenesis is not established; the pathophysiology of KMS is generally presumed to be that of platelet trapping by abnormally proliferating endothelium within the hemangioma. The mortality rate is high due to the coagulopathy. Many treatments have been tried showing different results. The objective of article is to provide a comprehensive review of KMS and give an up-to-date summary of treatment options.The clinical presentation of our sixteen cases of KMS, laboratory findings, vascular pathology, and pathophysiology will also be discussed.

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The function of leukocytes is dependent on a complex network of proteins. Many antibodies against leukocyte molecules have provided powerful analyzing tools, but each antibody had different associated nomenclatures. To bring order to the chaos, The Human Leukocyte Differentiation Antigens (HLDA) Workshops were initiated in 1982. The HLDA initiative has served immunology, hematology, and pathology well, and has supported research, diagnosis, and therapy through a universal nomenclature scheme. The scope of the workshops has been extended from leukocytes to other cell types including platelets, erythroid, dendritic, and non-hematopoietic cells. The 8th HLDA meeting, held in Adelaide, Australia, in December 2004, allocated 95 new CD designations and their numbers reached CD339. The acronym HLDA will be succeeded by HCDM (human cell differentiation molecules). This review briefly describes these CD antigens.

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We report here a case of Burkitt's lymphoma treated with R-CHOP regimen as salvage chemotherapy. A 13-year old boy was admitted due to abdominal distention and testicular swelling. He was diagnosed as having Burkitt's lymphoma stage III by pathological examination and clinical imaging. Because a perforation of the appendix and acute pancreatitis occurred, induction chemotherapy was discontinued. Rituximab was administered to prevent progression of lymphoma during treatment of the complications, and he achieved his first complete remission. Intensive chemotherapy was not able to be performed because of severe fatty liver and recurrent infection related to hypogammaglobulinemia. Eight cycles of R-CHOP regimen consisting of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone were performed as salvage therapy. The patient remains in complete remission after 14 months. R-CHOP regimen might be well tolerated for salvage therapy of Burkitt lymphoma with complications.

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Sjogren syndrome (SJS) in childhood rarely presents a symptom of xerosis of mouth or eye. Usually the patients of SJS in childhood show recurrent parotitis or asymptomatic hypergammaglobulinemia. We report a 14-yearold-girl with recurrent purpura and erythematous rash on her lower extremities. Serum IgG was 3, 724 mg/dl, anti SS-A and SS-B antibodies were positive. Biopsy on labial glands showing typical infiltration of numerous lymphocytes confirmed the diagnosis of SJS. A bone marrow specimen showed grape cells. Grape cells had been reported to be found in multiple myeloma or plasma cell neoplasms of adulthood. Grape cells reported in childhood are, to our knowledge, extremely rare. Accelerated production of immunoglobulin in autoimmune disease was considered to be the cause of the presence of grape cells.

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The patient was a 6-year-old girl with acute lymphoblastic leukemia (ALL). During induction chemotherapy, fever and thoracic pain occurred with increased plasma β-D-glucan and plasma Aspergillus DNA despite prophylactic administration of antibiotics and fluconazole. Thoracic computed tomography (CT) revealed an abscess and infiltration in the right S3 area, suggesting diagnosis of invasive pulmonary aspergillosis. Although administration of micafungin (MCFG) temporarily improved the symptoms, the lung abscess then spread with infiltration involving the right S4, S5, and S9 areas, which required additional administration of amphotericin B, itraconazole, and granulocyte colony-stimulating factor. With additional antifungal agents, plasma β-D-glucan and plasma Aspergillus DNA became negative, and thoracic CT showed improvement of the lung abscess and infiltration. Complete remission of ALL was sustained during the 6 months of chemotherapy interruption, and currently, additional chemotherapy has been resumed. Combination therapy with MCFG and other antifungal agents may be useful for treating refractory pulmonary aspergillosis with respect to efficacy and safety.

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Leukemic presentation of anaplastic large cell lymphoma (ALCL) is very rare. We here report a case of ALCL with Klinefelter syndrome, who developed an evolution to the leukemic phase. A 14-year-old male initially presented with an anterior mediastinal malignant teratoma, which contained immature teratoma and a yolk sac tumor. Complete resection of the tumor was performed after combination chemotherapy including cyclophosphamide, vincristine, pirarubicin and cisplatin. However, he complained of left shoulder pain six months after the operation. Computed tomography showed a soft tissue tumor with bone destruction in his left humerus. Biopsy specimens from the lesion identified the diagnosis of ALCL with expression of the cytokine receptor CD30 and anaplastic lymphoma kinase 1. Delayed puberty and chromosome analysis of bone marrow confirmed a diagnosis of ALCL with Klinefelter syndrome. Despite chemotherapy, he rapidly developed a leukemic phase in association with the progression of the disease.

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