Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
Volume 41, Issue 9
Displaying 1-13 of 13 articles from this issue
  • Satoru HAYASHI, Machiko OSHIDA, Teruo KIYOI, Seiji TADOKORO, Hirokazu ...
    2000 Volume 41 Issue 9 Pages 705-711
    Published: 2000
    Released on J-STAGE: July 28, 2009
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    Measurements of plasma glycocalicin (GC) and reticulated platelets (RP) have been reported to be useful for classifying thrombocytopenic disorders. However, there have been no reports comparing the clinical usefulness of the two methods. We measured GC and RP levels simultaneously in 39 patients with idiopathic thrombocytopenic purpura (ITP), 15 patients with aplastic anemia (AA), and 17 patients with hypoplastic thrombocytopenia (HypoT) due to chemotherapy. The GC index (GC level normalized for the individual platelet count) and the percentage of RP (%RP), a parameter of platelet life span, were very high (7.5±11.4 and 20.8±13.0%, respectively) in patients with ITP as compared with those of healthy subjects (1.3±0.5 and 7.9±2.5%, respectively). However, 6 AA patients and 14 HypoT patients, in whom platelet life span is thought to be normal, also had an elevated GC index, suggestive of a false positive result. The RP, a parameter of platelet production, was low in all AA and HypoT patients except for one in each case. However, the GC level, an additional parameter of platelet production, was normal in 4 AA and 8 HypoT patients, indicating that it is not a sensitive indicator. We conclude that the RP and %RP are more feasible markers of thrombopoiesis and platelet life span, respectively, than the GC level and GC index.
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  • Hiroki YAMAGUCHI, Mitsuhiro HASHIMOTO, Taro MIZUKI, Hiroyuki NAKAMURA, ...
    2000 Volume 41 Issue 9 Pages 712-717
    Published: 2000
    Released on J-STAGE: July 28, 2009
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    A 43-year-old man was admitted to our hospital because of hemophagocytic syndrome (HPS) in August, 1998. A CT scan, gallium scintigraphy, gastrofiberscopy and colonofiberscopy showed no evidence of malignant lymphoma. Virus-associated HPS was suspected because of an increased titer of anti-Epstein-Barr (EB) virus antibody (EBV VCA IgG 2,560×, EBV EA IgG 40×, EBNA 20×). The HPS resolved spontaneously for 40 hospital days, but two weeks into the period of HPS remission, the patient developed pain and marked swelling of the right thigh muscle, and pectoral, biceps brachii, quadriceps femoralis and masseter muscles. Otherwise, CT scan and gallium scintigraphy showed no abnormal findings. A biopsy of the right quadriceps femoralis muscle revealed non-Hodgkin's lymphoma with muscle infiltration. Immunohistologic examination confirmed T-cell type (CD3, CD43, CD45, CD45RO) lymphoma, and Southern blot analysis for T-cell receptor revealed a rearranged band. The lymphoma cells were negative for EBV genome monoclonality. The patient responded well to CHOP therapy and achieved a complete remission. This is considered a very rare case of T-cell lymphoma infiltrating multiple skeletal muscles following an episode of hemophagocytic syndrome.
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  • Reiko UENO, Jin TAKEUCHI, Tetsuo SHIMIZU, Takao KUMAGAI, Umihiko SAWAD ...
    2000 Volume 41 Issue 9 Pages 718-722
    Published: 2000
    Released on J-STAGE: July 28, 2009
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    A 54-year-old woman visited our hospital because of gingival bleeding on May 31, 1998. After hematological and bone marrow examinations, she was diagnosed as having acute promyelocytic leukemia (APL) and given all-trans retinoic acid (ATRA) therapy starting on June 1. Anti-cancer drugs were administered for 5 days from June 12 because of an increase in the number of APL cells. The patient developed fever on June 20, and nodular erythematous eruptions appeared on June 23. Sweet's syndrome was diagnosed from biopsy samples of the eruption. ATRA was therefore discontinued, and prednisolone was started on June 29. The fever and skin eruptions improved rapidly, and complete remission was obtained on July 13. Sweet's syndrome due to ATRA may be a partial form of retinoic acid syndrome, in which the differentiated leukemic neutrophils increase and invade various organs. However, Sweet's syndrome must be considered regardless of the WBC count because in this case the syndrome occurred even when the WBC count was not high.
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  • Tomoyuki HANDA, Katsuya YAMAMOTO, Jiro TADOKORO, Yasuko KIKKAWA, Shige ...
    2000 Volume 41 Issue 9 Pages 723-728
    Published: 2000
    Released on J-STAGE: July 28, 2009
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    We report a rare case of hypereosinophilic syndrome (HES) that developed to acute myeloblastic leukemia (AML). The patient, a 34-year-old man, presented with eosinophilia of unknown origin (white blood cells 38,200/μl with 74% eosinophils) and pericardial effusion, and was diagnosed as having HES with a normal karyotype. He received four cycles of combination chemotherapy including cyclophosphamide, cytosine arabinoside and vindesine, and thereafter remained in remission. After 12 years, he was referred to our hospital because of fever and malaise. On admission, CBC showed white blood cells 3,000/μl with 70% myeloblasts and 3% eosinophils. The bone marrow was hypercellular with 95% blasts, which were negative for myeloperoxidase (MPO) staining. Immunophenotype analysis revealed that the cells were positive for CD13, CD19, CD34, HLA-DR and cytoplasmic MPO. CD19-positive AML was diagnosed. Cytogenetic analysis showed 46, XY, t(6;21)(q13;q22), add(7)(q11) in 19 of 20 metaphase spreads. Rearrangement of the AML1 gene at 21q22 and fusion of the BCR/ABL gene could not be detected by fluorescence in situ hybridization analysis. The patient received combination chemotherapy and achieved a complete remission. Chromosome aberrations involving 7q as well as 21q22 suggested that the initial chemotherapy for HES might have been implicated in the pathogenesis of acute leukemia in this case.
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  • Yasushi NARIGASAWA, Yoko ONO, Shin-ichiro KURIYA
    2000 Volume 41 Issue 9 Pages 729-732
    Published: 2000
    Released on J-STAGE: July 28, 2009
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    A 37-year-old woman was admitted to our hospital in March 1995 because of high fever and cervical lymph node swelling. She had received prednisolone (PSL) therapy for autoimmune hemolytic anemia (AIHA). Laboratory examinations revealed increased serum levels of liver enzymes and C-reactive protein. Levels of rheumatoid factor and anti-nuclear antibody were within normal limits. On the basis of these criteria, she was diagnosed as having adult-onset Still's disease (AOSD). Although her fever ameliorated and physical symptoms disappeared immediately after a moderate dose of PSL, hemolysis recurred when attempts were made to reduce or withdraw the steroid. The patient is now receiving low-dose PSL therapy and is free of AIHA and AOSD.
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  • Kazutaka SUNAMI, Takayuki FUJIWARA, Chikamasa YOSHIDA, Soichiro FUJII, ...
    2000 Volume 41 Issue 9 Pages 733-738
    Published: 2000
    Released on J-STAGE: July 28, 2009
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    A 22-year-old man with non-Hodgkin's lymphoma (B-cell lymphoblastic lymphoma, Stage IVA) received chemotherapy and radiation therapy and achieved complete remission. He was admitted for allogeneic bone marrow transplantation (BMT) using a graft from his completely HLA-matched mother. Although he had HBV infection, allogeneic BMT was performed because he still had normal liver function and strongly requested the procedure. He developed both acute and chronic GVHD after the procedure, but showed no liver damage related to HBV. Treatment with lamivudine (150 mg/day) was started because the HBV-DNA level increased gradually after allogeneic BMT. Although the HBV-DNA then decreased gradually and there was no evidence of severe liver damage, the patient died following relapse of NHL. It seems that in this case, treatment of HBV with lamivudine may have prevented serious liver damage after allogeneic BMT. Therefore, allogeneic BMT may be done safely in patients with HBV infection if lamivudine is administered.
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  • Nobuhide KUSABA, Hiroshi YOSHIDA, Fumihiko OHKUBO, Yutaka SHIMOKAWA, M ...
    2000 Volume 41 Issue 9 Pages 739-744
    Published: 2000
    Released on J-STAGE: July 28, 2009
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    A 53-year-old woman developed fever and fatigue after eating raw oysters, and consulted a local clinic. She exhibited liver dysfunction and jaundice, and was therefore referred and admitted to our hospital. Hepatitis A was diagnosed based on virus markers, and palliative therapy was administered. After admission, the increased levels of transaminases and lactic acid dehydrogenase (LDH) normalized rapidly. However, marked thrombocytopenia was observed, and steroid pulse therapy was administered. Thereafter, idiopathic anemia developed, and a bone marrow puncture was performed. Bone marrow smears showed marked depletion of cells of the erythroblastic and megakaryocytic series. Parvovirus B19 infection was ruled out, and an additional steroid was administered. However, the patient's anemia and thrombocytopenia persisted. She suffered a prolonged period of cholestasis and died of acute renal failure. At autopsy, the bone marrow revealed severe erythroblastopenia. A diagnosis of thrombocytopenia due to an autoimmune mechanism was made on the basis of elevated levels of platelet-associated immunoglobulin G (PAIgG) and immune complex. Furthermore, the advanced anemia was complicated by concurrent hemolysis. This case may provide information useful for clarifying the pathogenesis of hematopoietic disorders complicated by hepatitis.
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  • Etsuko YAMAZAKI, Kouji OGAWA, Hiroshi HARANO, Heiwa KANAMORI, Yoshiaki ...
    2000 Volume 41 Issue 9 Pages 745-749
    Published: 2000
    Released on J-STAGE: July 28, 2009
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    We report a case of acute myelocytic leukemia (AML) showing a chromosomal abnormality, ins(21;8), with AML1/MTG8 chimeric mRNA. The patient, a 73-year-old woman, was admitted to our hospital because of AML relapse. Bone marrow aspiration showed 44% blasts and ins(21;8)(q12;q13q22) by cytogenetic study. Moreover, the size of chimeric AML1/MTG8 mRNA detected by RT-PCR in this case was shorter than that of previously reported. The patient was diagnosed as having relapse of AML (M2), but achieved complete remission with DCP therapy. Four months later, extramedullary relapse occurred, and this was followed five months later by bone marrow relapse. However, the patient again achieved complete remission. Most cases of AML1/MTG8 fusion gene are caused by t(8;21), and only very rarely by ins(21;8). In this case, the AML1/MTG8 fusion gene is thought to have been involved in the onset of leukemia.
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  • Yasuo HIRAYAMA, Tadanori NAGAI, Hidetoshi OHTA, Ryuzo KOYAMA, Takuya M ...
    2000 Volume 41 Issue 9 Pages 750-754
    Published: 2000
    Released on J-STAGE: July 28, 2009
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    A 62-year-old woman was admitted because of leukocytosis, systemic lymph node swelling and erythroderma. Laboratory data disclosed a WBC count of 15,600/μl (CD4-positive cells: 91%). CD25 and HTLV-1 were negative. A skin biopsy revealed the involvement of T cells. These data and findings were consistent with a diagnosis of Sézary syndrome. Although the patient was treated with 2 courses of CHOP, the leukocyte count did not decrease. We then treated the patient orally with etoposide (25 mg/day) and methotrexate (10 mg/week), and this resulted in reduction of the leukocyte count and lymph node swelling, and amelioration of the erythroderma. As Sézary syndrome is an extremely rare disease, it is worthwhile reporting cases like the present one, where therapy was successful.
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  • Yasunobu TAKEOKA, Takahisa YAMANE, Ki-Ryang KOH, Kensuke OHTA, Hirohis ...
    2000 Volume 41 Issue 9 Pages 755-760
    Published: 2000
    Released on J-STAGE: July 28, 2009
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    A 57-year-old woman was admitted to our hospital with suspected leukemia in September 1999. In 1990, systemic erythema had occurred, and mycosis fungoides (MF) had been diagnosed by skin biopsy. Interferon-γ therapy had not been effective, and the erythema had disappeared after treatment with psoralen and ultraviolet A (PUVA) therapy (1.46 J/cm2). The patient had subsequently done well with a course of topical steroids. On admission this time, the WBC count was 1,600/μl with 6% blasts. The total nucleated cell count in a bone marrow aspirate was 43.1×104l, of which 86.2% were peroxidase-positive blasts. Acute myelocytic leukemia (AML) was diagnosed. Chromosomal analysis demonstrated abnormalities of 48, XX, +4, +8, +add(10)(p11), add(11)(q23) in 10 of 20 cells, and 51, idem, +6, +8, +21, +mar in 8 cells with mixed-lineage leukemia gene rearrangement. Therapies (radiation, chemotherapy and PUVA) for MF, and the altered immune response seen in patients with this disease, especially in the more advanced stages, collectively termed cutaneous T-cell lymphoma (CTCL), suggest that such patients may be at increased risk of a second primary malignancy. To our knowledge, AML has been reported in 8 MF patients including the present one. Attention should be given to the possibility of MF terminating in AML.
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  • Riko KAWANO, Mitsuhide KIMURA, Yumi NOGUCHI, Tatsuro GOTO, Hiroshi TAK ...
    2000 Volume 41 Issue 9 Pages 761-763
    Published: 2000
    Released on J-STAGE: July 28, 2009
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    We describe a 46-year-old HLA-homozygous female patient with CML who received a bone marrow transplant from her son, who had two HLA (A, B) loci mismatch. After conditioning with total body irradiation plus cyclophosphamide, the patient received 4.8×108 bone marrow cells/kg. Cyclosporin and short-term methotrexate were used for GVHD prophylaxis. She successfully established rapid engraftment with no acute GVHD, and later developed chronic but mild GVHD. Family members with two HLA loci mismatch may be considered as candidate donors for “hetero to homo” bone marrow transplantation.
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  • Mitsuhide KIMURA, Yuji YUFU, Riko KAWANO, Tatsuro GOTO, Hiroshi TAKATS ...
    2000 Volume 41 Issue 9 Pages 764-767
    Published: 2000
    Released on J-STAGE: July 28, 2009
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    Thirty cases of childhood acute lymphoblastic leukemia (ALL) with a near haploid karyotype (<30 chromosomes) have been reported so far. However, despite a few cases of severely hypodiploid (30∼39 chromosomes) ALL, no near haploid cases have been reported in adult patients. Here, we describe a 64-year-old woman with ALL (L2, CD10+ 19+ 34+ HLA-DR+) presenting a near haploid karyotype of 27, X, +X, +6, +10, +21/54, idem×2. She died of septic shock during complete remission 6.5 months after the diagnosis.
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