Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
Volume 42, Issue 11
Displaying 1-12 of 12 articles from this issue
  • Toru TEZUKA, Kanji SUGITA, Hiroki SATO, Kanako UNO, Naoko YAMAKAWA, Ku ...
    2001 Volume 42 Issue 11 Pages 1096-1100
    Published: 2001
    Released on J-STAGE: July 28, 2009
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    Human parvovirus B19 (HPV-B19) infection is known to frequently induce aplastic crisis in patients with hemolytic anemia, but rarely causes hematological disorders in healthy subjects. We report a 5-year-old boy who showed transient myelodysplasia associated with HPV-B19 infection. He was admitted with severe nasal bleeding, and laboratory data showed marked thrombocytopenia (1,000/μl), anemia (Hb: 8.4 g/dl), and mild leukopenia (3,000/μl). A bone marrow smear revealed myelodysplastic changes in three cell lineages, but no giant proerythroblasts or megakaryocytes. The pancytopenia and myelodysplastic changes were resolved spontaneously within a week without any medication other than transfusion of red blood cells and platelets. Serological examination revealed an elevation of IgG antibody against HPV-B19 on days 15 and 120 and its subsequent decrease thereafter, although HPV-B19-specific DNA was not detected in the serum at onset. The clinical course and laboratory data suggest an etiologic role of HPV-B19 infection in the occurrence of transient myelodysplasia.
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  • Jun OGINO, Chikako KAWAKATSU, Akira HIRASAWA, Tadatsugu SATO, Shunji K ...
    2001 Volume 42 Issue 11 Pages 1101-1104
    Published: 2001
    Released on J-STAGE: July 28, 2009
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    A 43-year-old man with macrohematuria and anuresis was admitted, and diagnosed as having bladder tamponade due to coagulates. Abdominal ultrasonography and computed tomography revealed bilateral renal tumors. Bilateral renal arteriography showed hypovascular lesions. Percutaneous needle biopsy of the left renal tumor was performed, and the final diagnosis was non-Hodgkin's lymphoma (diffuse mixed, B cell type, CSIIA). After six courses of chemotherapy, the tumor lesions were markedly reduced, and at present there is no evidence of recurrence.
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  • Kazuhiro YAGO, Mitsuru ITOH, Hideto SHIMADA
    2001 Volume 42 Issue 11 Pages 1105-1110
    Published: 2001
    Released on J-STAGE: July 28, 2009
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    We report a case of Epstein-Barr virus-associated lymphoproliferative disorder (EBV-LPD) after allogeneic bone marrow transplantation (allo-BMT) from a partially mismatched related donor for acute lymphocytic leukemia, which was treated successfully by donor lymphocyte infusion (DLI). A 54-year-old woman in first complete remission from acute lymphocytic leukemia received an unmanipulated bone marrow transplant from an HLA-A 1-locus-mismatched sibling donor after precon-ditioning with cyclophosphamide and total body irradiation. Prophylaxis against graft-versus-host disease (GVHD) was done with tacrolimus and short-term methotrexate. Skin GVHD (grade I) occurred on day 36, but this subsided spontaneously without treatment. On day 61, rapidly progressive cervical lymphadenopathy with fever developed. Lymph node biopsy revealed lymphoid cell proliferation, which was positive for LCA, L26 and LMP-1. A diagnosis of EBV-LPD was made. After withdrawal of the tacrolimus, DLI (1×106 CD3 cells/kg) resulted in remission. This case suggests that even in the absence of risk factors such as severe GVHD, intensive immunosuppressive therapy and ATG administration, allo-BMT from an HLA 1-locus-mismatched related donor can be complicated by EBV-LPD, and that reduction of immunosuppressive therapy and DLI can be an effective treatment for it.
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  • Masahiro YASUI, Takayuki OKAMURA, Naoki SAKATA, Masami INOUE, Keiko YA ...
    2001 Volume 42 Issue 11 Pages 1111-1116
    Published: 2001
    Released on J-STAGE: July 28, 2009
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    We report two boys with chronic active Epstein-Barr virus infection (CAEBV) refractory to conventional chemotherapy, who received HLA-mismatched allografts of CD34-positive progenitor cells from their fathers. One patient developed veno-occlusive disease (VOD) of the liver on day 18 after transplantation and died on day 26. The other patient received the allograft during partial remission. Although he suffered recurrent infections due to Streptococcus viridans, he is now doing well 23 months after transplantation. CAEBV refractory to chemotherapy is considered to be a fatal EBV-infected T/NK-cell lymphoproliferative disease, and our experiences suggest that CD34-positive progenitor cell transplantation for patients with CAEBV lacking HLA-matched donors may be a feasible and useful treatment. However, the timing of transplantation is considered to be critical, and should be performed when the patient is in good clinical condition.
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  • Kei MURAYAMA, Atsushi SHIBUYA, Saori ISHII, Nozomu SASAKI
    2001 Volume 42 Issue 11 Pages 1117-1121
    Published: 2001
    Released on J-STAGE: July 28, 2009
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    Priapism is an uncommon clinical symptom in children with chronic myelogenous leukemia (CML). Here we report a 14-year-old boy with this symptom, which had appeared 4 days prior to hospitalization. Peripheral blood examination revealed a leukocyte count of 510,000/μl, (87% neutrophils, 3% eosinophils, 6% basophils, and 1.6% lymphocytes), a hemoglobin level of 6.5 g/dl and a platelet count of 640,000/μl. Karyotype analysis revealed the Ph1 chromosome and myeloid hyperplasia in the bone marrow. The patient was diagnosed as having chronic myelogenous leukemia (CML) complicated by priapism. In an unsuccessful attempt to alleviate and improve the priapism, urokinase was injected and hydroxyurea was administered for the CML. Angiography confirmed the presence of venous return from the scrotum, and embolization of the bilateral internal pudendal arteries was performed to reduce the amount of inflow. Although this relieved the patient of his pain and prevented penile necrosis, the patient's future sexual potency was sacrificed. Selective embolization of the pudendal arteries can be one of the most effective ways of treating intractable priapism, if angiography confirms the presence of venous return from the penis.
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  • Manabu SODA, Kensuke USUKI, Yuko ADACHI, Hiroshi KAZAMA, Seiko IKI, Ak ...
    2001 Volume 42 Issue 11 Pages 1122-1127
    Published: 2001
    Released on J-STAGE: July 28, 2009
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    An 18-year-old woman was admitted to our hospital because of severe anemia on October 16, 1999. Laboratory data included hemoglobin 3.5 g/dl, reticulocytes 2,200/μl, WBC 3,500/μl, and Plt 38.5×104l. Bone marrow aspiration showed a normocellular marrow with severe erythroid hypoplasia, suggesting a diagnosis of pure red cell aplasia. Methylprednisolone pulse therapy was started on October 20, but there was no response. Administration of cyclosporine A (CyA; 400∼450 mg) was begun on November 1, but again there was no response. Antithymocyte globulin (ATG; 800 mg/day for 5 days, 15 mg/kg) was started from December 1 in addition to prednisolone (60 mg/day) and CyA (450 mg/day). On day 7 of ATG therapy, the reticulocyte count began to increase, and reached a peak of 32.6×104l on day 20. The patient's hemoglobin level started to increase on day 13, and reached 8.5 g/dl on day 27. A complete response has been maintained up to the time of writing, and the hemoglobin level was 11.9 g/dl on December 14, 2000. This is the first detailed Japanese case report of successful treatment of pure red cell aplasia using ATG.
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  • Kiyotaka KAWAUCHI, Toshie OGASAWARA, Masako YASUYAMA, Shinichiro OHKAW ...
    2001 Volume 42 Issue 11 Pages 1128-1133
    Published: 2001
    Released on J-STAGE: July 28, 2009
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    A 65-year-old man was admitted to our hospital in September 1997 because of back pain and renal dysfunction. He was diagnosed as having multiple myeloma due to the presence of IgDλ monoclonal gammopathy and diffuse infiltration of plasma cells in the bone marrow. The patient achieved a partial response to DMVM-IFN-α combination therapy. His condition worsened in December 1998, but was ameliorated by VAD therapy. The patient's left testis became enlarged in December 1999, and an orchiectomy was performed. The normal testicular cells had been entirely displaced by myeloma cells comprising typical plasma cells and large lymphoid cells. Pleural, mediastinal, spinal, and right testicular involvement with myeloma subsequently developed. Despite attempts to treat the patient with more than one type of combination therapy, his condition worsened progressively, and he died in June 2000. Reports of IgD myeloma with testicular involvement are rare. The histopathology of our patient's resected testis, i.e. the two myeloma cell-plasmacytoid and lymphoid cell components showing differential immunostaining, was unique.
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  • Keiki KAWAKAMI, Yasuyuki WATANABE, Fumihiko MOMMA, Hideki NOMURA
    2001 Volume 42 Issue 11 Pages 1134-1138
    Published: 2001
    Released on J-STAGE: July 28, 2009
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    We report a case of angioimmunoblastic T-cell lymphoma (AITL) in a 48-year-old Japanese male. Characteristic clinical manifestations of AITL such as fever, weight loss, skin rash, general lymphadenopathy, Coombs test positivity, and polyclonal hypergammaglobulinemia were present. Histopathologically, the nodal architecture was preserved and germinal centers were, if anything, hyperplastic, which was unusual for AITL. Diagnostic clear cells were absent. Intrafollicular tingiblebody macrophages were markedly increased. In the interfollicular zone, proliferation of UCHL-1+ immunoblasts was evident, and high-endothelial postcapillary venule-type vessels were increased. The T-cell receptor gene (Cβ1) was clonally rearranged. Based on these findings, a diagnosis of AITL with hyperplastic germinal centers was made. Six courses of CHOP were administered and no signs of relapse were noticed about two years after diagnosis.
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  • Etsuko YAMAZAKI, Hiroshi HARANO, Shin FUJISAWA, Shouichi KOBAYASHI, Ko ...
    2001 Volume 42 Issue 11 Pages 1139-1141
    Published: 2001
    Released on J-STAGE: July 28, 2009
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    We report a Japanese family with Sebastian platelet syndrome. Twenty-one thrombocytopenic patients exhibited giant platelets and inclusion bodies in their granulocytes. They were thought to be related because they bore the same surname and lived within a localized area. None of them had additional clinical findings peculiar to Fechtner syndrome. Ultrastructural studies of the granulocytes were performed on four patients. The inclusion bodies in the granulocytes were different from those found in May-Hegglin anomaly, and consisted of ribosome clusters and rough endoplasmic reticula.
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  • Kinuyo KANEDA, Kensuke KOJIMA, Katsuji SHINAGAWA, Fumihiko ISHIMARU, K ...
    2001 Volume 42 Issue 11 Pages 1142-1144
    Published: 2001
    Released on J-STAGE: July 28, 2009
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    A 40-year-old man was admitted to our hospital because of a tendency to bleed. A diagnosis of idiopathic thrombocytopenic purpura was made, and oral prednisolone (1 mg/kg/day) was adminstered immediately. Three days after admission, hemorrhagic skin rashes highly suggestive of varicella appeared. The oral prednisolone was discontinued, and intravenous γ-globulin (400 mg/kg/day for 3 days) and aciclovir (750 mg/day for 7 days) were started. The platelet count increased to 254,000/μl over the next five days, and the skin rashes associated with varicella subsided within a week. We suggest that, in a minority of patients with varicella zoster infection, thrombocytopenia can precede the typical skin rashes, so a search for possible underlying viral infection should be made, and if necessary, immediate treatment started.
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  • Akiko FUKUTA, Takeshi HARA, Hisashi TSURUMI, Hisataka MORIWAKI
    2001 Volume 42 Issue 11 Pages 1145-1147
    Published: 2001
    Released on J-STAGE: July 28, 2009
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    A 47-year-old man was admitted to our hospital with subcutaneous nodules on the bilateral lower legs and disseminated intravascular coagulation (DIC). Peripheral blood examination revealed leukocytosis with an increase of mature eosinophils, thrombocytopenia and abnormal coagulation. Bone marrow aspiration revealed an increased eosinophil count, and a diagnosis of hypereosinophilic syndrome (HES) was made. Prednisolone (PSL) therapy was not effective. Subsequent methylPSL pulse therapy followed by PSL brought about a transient improvement of the HES and DIC, but after reduction of the PSL, the HES worsened. After addition of cyclosporin A to the PSL, however, the HES improved and did not worsen.
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