Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
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Volume 41, Issue 6
Displaying 1-16 of 16 articles from this issue
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  • CD34+ cell dose and hematologic recovery in allogeneic peripheral blood stem cell transplantation
    Hitoshi SAWADA, Atsushi WAKE, Yoshihiro YAMASAKI, Yoichiro IZUMI
    2000 Volume 41 Issue 6 Pages 500-506
    Published: 2000
    Released on J-STAGE: July 28, 2009
    DOIhttps://doi.org/10.11406/rinketsu.41.500
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    Allogeneic peripheral blood stem cell transplantation (Allo-PBSCT) has been performed as an alternative to bone marrow transplantation (BMT). Here we report poor mobilization with granulocyte-colony stimulating factor (G-CSF) and engraftment kinetics in Allo-PBSCT. Sixteen patients (aged 6∼61 yr, median 34 yr) received allogeneic peripheral blood stem cells from related donors (aged 15∼68 yr, median 37 yr) after myeloablative therapy. Nine of the patients had standard-risk disease and 7 had high-risk disease. The donors received G-CSF at a dose of 10 μg/kg/day by subcutaneous injection for 4 to 6 days. Peripheral blood stem cells were subsequently collected in 1 to 3 aphereses and infused immediately. All patients received G-CSF after transplantation. Fifteen patients underwent Allo-PBSCT and one underwent Allo-PBSCT plus BMT. The mean number of CD34+ cells infused in the 15 Allo-PBSCT patients was 6.32×106/kg (range 1.28∼14.20). The outcomes were compared with 9 identically treated patients who underwent Allo-BMT. The median times until engraftment for neutrophils >500/μl and platelets >20,000/μl were 14 (range 10∼17) and 15 (range 11∼50) days in the Allo-PBSCT group and 17 (range 13∼29) and 20 (range 16∼160) days in the Allo-BMT group, respectively (p=0.0177 and p=0.003). Three donors were considered to have poor mobilization (<2×106 CD34+ cells/kg of the recipient); two of them yielded 1.28 and 1.78×106 CD34+ cells/kg in 3 apheresis procedures. The patients who received cells from these donors showed prompt neutrophil engraftment, but one showed delayed platelet engraftment and another died of grade IV acute GVHD before reaching 20,000 platelets/μl. An additional bone marrow harvest was necessary from one donor because of poor mobilization (0.17×106 CD34+ cells/kg). Thus, Allo-PBSCT results in more rapid engraftment. It will be necessary to clarify the minimum CD34+ cell dose for complete engraftment in a larger series of trials.
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  • Diffuse large B-cell lymphoma showing CNS invasion by CD30-positive multinuclear giant cells mimicking the clinical features of progressive multifocal leukoencephalopathy
    Keiko ANDO, Takashi SHIMAMOTO, Shigefumi HAYASHI, Yoshikazu ITO, Yoshi ...
    2000 Volume 41 Issue 6 Pages 507-512
    Published: 2000
    Released on J-STAGE: July 28, 2009
    DOIhttps://doi.org/10.11406/rinketsu.41.507
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    We report a 55-year-old woman with diffuse large B-cell lymphoma showing central nervous system (CNS) infiltration by CD30-positive lymphoma cells. The patient was admitted with pleural effusion, ascites and a large mass in the abdominal cavity. Southern blot analysis of DNA extracted from the ascites revealed IgJH rearrangement, and therefore she was initially diagnosed as B-cell neoplasia. She received combined chemotherapy (DICE and CHOP regimens), and achieved a transient clinical response. Three months later, she developed various neurological abnormalities, and brain magnetic resonance imaging revealed diffuse infiltration of the cerebral white matter. We considered the possibility of CNS involvement by the lymphoma or progressive multifocal leukoencephalopathy (PML), and began a course of anti-virus therapy and radiation therapy. Because multiple lumbar punctures demonstrated large multinuclear lymphoma cells in the cerebrospinal fluid, a diagnosis of metastatic CNS lymphoma was made. Immunohistochemistry revealed that these lymphoma cells were reactive with anti-CD30 antibody. Although the radiation therapy was temporarily effective against the CNS involvement, the patient died of systemic invasion of the lymphoma cells. The final diagnosis was diffuse large B-cell lymphoma on the basis of pathologic findings, immunohistochemistry, and Southern blot analysis using a mesenteric lymph node obtained at autopsy. Cytospin preparations and immunohistochemistry of specimens obtained from frequent lumbar punctures were useful for differentiating CNS lymphoma from PML.
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  • Complete remission of plasmablastic IgD λ multiple myeloma induced by continuous infusion of low-dose cytarabine and etoposide
    Kenji FUKUNO, Hisashi TSURUMI, Toshiki YAMADA, Masami OYAMA, Hisataka ...
    2000 Volume 41 Issue 6 Pages 513-518
    Published: 2000
    Released on J-STAGE: July 28, 2009
    DOIhttps://doi.org/10.11406/rinketsu.41.513
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    A 67-year-old man was admitted because of thrombocytopenia in May 1998. His white blood cell count was 4,900/μl with 3.5% blasts. Laboratory findings were as follows: hemoglobin level, 10.1 g/dl; platelet count, 1.8×104/μl; ALT, 56 IU/l; LDH, 3,570 IU/l; IgG, 653 mg/dl; IgA, 64 mg/dl; IgM, 49 mg/dl; IgD, 674 mg/dl. Serum immunoelectrophoresis confirmed IgD λ M-component. Bone marrow aspiration showed 79.2% myeloma cells expressing a mostly plasmablastic morphology. No mature plasma cells were found in the bone marrow. The patient received a continuous drip infusion of 20 mg/body cytarabine (Ara-C) and 50 mg/body etoposide (VP-16) for 7 days. No plasmablastic myeloma cells were detected, but 2.1% mature plasma cells were found in his bone marrow on day 20. On day 18 his platelet count exceeded 10.8×104/μl, and the serum IgD level fell to 210 mg/dl. Therapy consisting of melphalan, methylprednisolone and vincristine was started from day 23. No IgD λ M-component was detectable by serum immunoelectrophoresis seven months after the diagnosis of multiple myeloma. The patient has been in complete remission as of April 2000.
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  • Subcutaneous panniculitic T-cell lymphoma with chromosomal abnormalities and large granular lymphocytes morphology
    Yumiko MIZUTANI, Kikue IWAMASA, Junko ARAI, Ikuya SAKAI, Masaki YASUKA ...
    2000 Volume 41 Issue 6 Pages 519-523
    Published: 2000
    Released on J-STAGE: July 28, 2009
    DOIhttps://doi.org/10.11406/rinketsu.41.519
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    A 72-year-old woman was admitted because of a subcutaneous hip tumor. A biopsy specimen of the tumor showed a mixture of medium-sized and large lymphocytes infiltrating the subcutaneous fat tissue with a lobular panniculitis-like pattern—a histologic feature of subcutaneous panniculitic T-cell lymphoma (SPTCL). May-Grünwald-Giemsa-stained cytospin slides of freshly isolated neoplastic cell explants showed that the cells had the characteristics of large granular lymphocytes. Immunophenotypic analysis showed that the cells expressed CD56—a natural killer-associated antigen—as well as the cytotoxic T-cell phenotype CD3+ CD4- CD8+. Southern blot analysis revealed rearrangement bands of the TCR-β chain gene. Chromosome analysis showed complex abnormalities including t(1;6)(q11;p21). The present case may shed some light on the origin and pathogenesis of SPTCL.
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  • Genetic analysis of a patient with dyskeratosis congenita
    Toshimi YOSHIMOTO, Yasuhide YANABE, Tomoyuki MIZUKAMI, Fuminari ISHIBA ...
    2000 Volume 41 Issue 6 Pages 524-529
    Published: 2000
    Released on J-STAGE: July 28, 2009
    DOIhttps://doi.org/10.11406/rinketsu.41.524
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    Dyskeratosis congenita (DKC) is a rare inherited disease characterized by reticulated pigmentation of the skin, nail dystrophy and oral leukoplakia. More than 90% of DKC cases are inherited as an X-linked recessive trait. Half the patients develop progressive pancytopenia by the age of 11 yr, and this is the leading cause of death. We experienced a 11-year-old boy with the above symptomatic triad of DKC, complicated by progressive pancytopenia as well as cerebellar ataxia. Genetic analysis of mRNA from his cultured peripheral lymphocytes revealed a missense mutation resulting in substitution of 1,150 C with T in the DKC1 gene. This is identical to the mutation reported by Knight et al. to be prevalent in X-linked cases of DKC (11 out of 21 patients). Existence of the identical mutation in Japan suggests that this mutation has been selected on the basis of not only the DNA structural sequence of dyskerin, but also its biological function. We report the detailed clinical course of this Japanese DKC patient with a mutation in the DKC1 gene, and describe the results of genetic analysis.
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