Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
Volume 33, Issue 1
Displaying 1-16 of 16 articles from this issue
  • Kimio TANAKA, Miho TAKECHI, Chiharu SHIGETA, Nobuo OGUMA, Nanao KAMADA ...
    1992 Volume 33 Issue 1 Pages 1-10
    Published: 1992
    Released on J-STAGE: April 24, 2009
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    Eight cases with Ph1 positive acute leukemia (7 of acute lymphocytic leukemia: ALL, and one of acute myelocytic leukemia: AML) were studied molecular biologically to identify location of breakpoints on BCR gene in each patient. Six of the 8 patients (5 of ALL and 1 of AML) had rearrangements at bcr (M-BCR) region. Their locations of the breakpoint in M-BCR were similar to those of 59 chronic myelocytic leukemia patients. One of the remaining two patients had gene rearrangements at m-BCR-1 region in BCR intron 1, and the last patient did not have gene rearrangements at any site of m-BCR-1 and IgL Cλ region. Two cases had gene deletion at either 3' or 5' side of the bcr. A patient with bcr rearrangement was also analyzed by PCR method with reverse transcriptase (RT-PCR) and had simultaneous expressions of bcr3-abl and bcr2-abl chimeric mRNAs. These results indicate that Ph1 positive acute leukemia have heterogenous characterisitics in terms of the molecular biology. The molecular analysis will help for classifing the leukemic types and for elucidating the pathogenesis in Ph1 positive acute leukemia.
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  • Seiji KOJIMA, Yuji MIYAJIMA, Minoru FUKUDA, Takaharu MATSUYAMA, Hirosh ...
    1992 Volume 33 Issue 1 Pages 11-16
    Published: 1992
    Released on J-STAGE: April 24, 2009
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    Twenty-seven patients with aplastic anemia (20 severe: 7 moderate) were treated with combined immunosuppression consisting of antilymphocyte globulin (ALG: Ahlbulin, Green Cross Co., Osaka, Japan) and high-dose methylprednisolone. Danazol or meptiostane was administered concurrently for at least 3 months. Ten of 27 patients had sustained improvement in hematopoiesis within 3 months of treatment. Three patients with hematological response had a recurrence of pancytopenia 12∼36 months after the combined immunosuppressive therapy. Six patients died due to fungal pneumonia (2), hepatic failure (2), interstitial pneumonitis (1) and complication following allogeneic bone marrow transplantation (1). By life table analysis, the survival rate for all patients was 76±8% at 4 years, with 70±10% survival rate for patients with severe aplastic anemia and 100% for patients with moderate aplastic anemia. The factors predicting the good response to the therapy were a longer interval from diagnosis to the therepy and higher counts of platelet and reticulocyte at admission.
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  • Fumio MARUYAMA, Teruo INO, Hitoshi MIYAZAKI, Toshiyuki NOMURA, Koichi ...
    1992 Volume 33 Issue 1 Pages 17-23
    Published: 1992
    Released on J-STAGE: April 24, 2009
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    We analysed immunoglobulin (Ig) gene rearrangements in 28 patients with multiple myeloma by Southern hybridization method. We used 5 probes which cover Cκ and κ de loci of Ig light chain κ gene, and JH, 5'Sμ and Sγ3 loci of Ig heavy chain gene. In 11 out of 12 patients with κ-producing myeloma, DNA rearrangements were observed using Cκ probe. Among them, κde region was rearranged in 7 patients and kept germline configuration in 4 patients. In all of 14 patients with λ-producing myeloma, Cκ region was deleted and κ de region was rearranged. 5'Sμ-probe was very useful for detecting class switch recombination, and furthermore by using Sγ-probe together, Sμ-Sγ joining could be detected. In all of 10 patients with γ-producing myeloma, 5'Sμ and Sγ-probes detected the rearranged band of the same size on at least 1 allele, which suggested the presence of Sμ-Sγ joinings. In 8 of 10 patients with Bence-Jones myeloma, 5'Sμ-probe detected rearranged bands and the presence of class switch recombinations were suggested as observed in other Ig secretory myelomas. In other 2 patients with Bence-Jones myeloma, non-functional class switch recombinations were detected. The results of this study indicated that genotypes corresponded well to phenotypes in multiple myeloma, and further analysis in other types of B cell malignancies will be interesting.
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  • Ichiro TSUKIMOTO, Junichi MATSUI, Hiroko IWASHITA, Katsuyoshi SHIGETA, ...
    1992 Volume 33 Issue 1 Pages 24-29
    Published: 1992
    Released on J-STAGE: April 24, 2009
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    To elucidate the usefulness in prediction of hypersensitive reactions of L-asparaginase (L-asp), anti-L-asp IgG antibody was measured by two kinds of enzyme-linked immunosolvent assay (ELISA) with improved microplate method and original immunobeads method. The correlation coefficient of both methos matched very well (R=0.949). Nine out of the 10 patients who were treated with L-asp for the first time showed the elevation of the anti L-asp IgG antibody and returned to normal range within 9 months thereafter. Twenty-two out of 26 patients (84.6%) who had ever developed hypersensitive reactions showed the positive anti-L-asp antibody. There was no positive correlation between the anti-L-asp titer and the severity of the hypersensitive reactions. We concluded that the ELISA with microplate was simple and useful methods to determined the anti-L-asp IgG antibody.
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  • Masamichi MINESHITA, Sinsuke KAYASHIMA, Makoto MAKISHIMA, Fumihiko KIM ...
    1992 Volume 33 Issue 1 Pages 30-35
    Published: 1992
    Released on J-STAGE: April 24, 2009
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    A 36-year-old woman with disseminated intravascular coagulation syndrome (DIC) was admitted to the Third Department of Internal Medicine of the National Defense Medical College Hospital. Even after she recovered from DIC, she had a low level of C1-inactivator. Her past history, family history and family study revealed that she and her family showed hereditary angioneurotic edema (HANE). There have been no reports of HANE associated with DIC. She had two HANE attacks after discharge. We used nafamostat mesilate, a strong inhibitor of activated first complement component, to treat the attacks. She obtained subjective relief and her serological data were improved. Nafamostat mesilate was considered effective for HANE attack.
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  • Chien-yu SU, Nozomi ARAI, Masanori UMEDA, Tatsuo SHIRAI
    1992 Volume 33 Issue 1 Pages 36-41
    Published: 1992
    Released on J-STAGE: April 24, 2009
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    We report herein a 61-year-old man with chronic cold agglutinin disease which occurred after a diagnosis of aplastic anemia. The patient's pancytopenia was recognized upon visiting a local doctor because of high fever and cough on December 21, 1985. He was subsequently admitted to our hospital because of anasarca on January 31, 1986, and was diagnosed as having aplastic anemia. He was treated with prednisolone, and was discharged after his anemia improved. He was readmitted on October 23, 1988, because of icterus. Laboratory data on the patients second admission revealed increased reticulocyte count, hyperplastic bone marrow with a predominance of erythroblasts, increased serum indirect bilirubin, increased serum LDH1 value and decreased serum haptoglobin. Moreover, cold agglutinin titer was increased, anti-IF antibody was positive, and anti-IgM antibody was recognized with direct anti-globulin test. There was no precedent infection such as mycoplasma pneumonia or infectious mononucleosis. Hence, this patient was diagnosed as having chronic cold agglutinin disease.
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  • Shuji ONISHI, Susumu OKUBO, Tatsunori MATSUZAKI, Tomoko ISHIDA, Kojiro ...
    1992 Volume 33 Issue 1 Pages 42-47
    Published: 1992
    Released on J-STAGE: April 24, 2009
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    Here reported are two cases with neonatal alloimmune thrombocytopenia (NAITP). In our 2 cases, anti-HLA antibodies, i.e. anti-HLA-A24+B51 and anti-HLA-Bw61+Cw3, respectively, were detected in the mother's sera, which reacted to each patient's lymphocytes and were presumed to have been contributive to NAITP. The thrombocytopenia, found on routine laboratory examination, recovered within 1 to 3 weeks without any therapy in both cases. Further, the relationship of platelet count and PAIgG in neonatal babies (umbilical cord blood) to anti-HLA antibodies in their mothers were examined in 284 pairs. Thrombocytopenia less than 150,000/μl was found in 16/284 babies (5.6%), 3 of the 16 (1.1% of the total) being positive for anti-HLA, and one positive for PAIgG. Four (1.4%) of the 284 babies were positive for PAIgG, one of whom was thrombocytopenic, and one positive for anti-HLA. Anti-HLA antibody was found in 25/284 mothers (8.8%). Nevertheless, none of the pairs showed positive reactions in all of three tests. It seems that passive trasfer of maternal anti-HLA antibodies to the fetus is not itself sufficient to cause thrombocytopenia. On the other hand, some NAITP cases may have been overlooked because of their mildness.
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  • Takeshi TERASHIMA, Kazunori TOMINAGA, Morifumi TUJI, Hitoshi TAKEUCHI, ...
    1992 Volume 33 Issue 1 Pages 48-52
    Published: 1992
    Released on J-STAGE: April 24, 2009
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    A 75-year old man was admitted to our hospital by complaining of his left supraclavicular lymphadenopathy. Physical examination also revealed splenomegaly and cervical lymphadenopathy. An abdominal CT scan showed paraaortic lymphadenopathy and splenomegaly with multiple low density area. A pathological diagnosis of Ki-1 lymphoma was made by the biopsied supraclavicular lymph node because the node consists of large cells with clear cytoplasms and conspicuous nucleoli and large cells were positive for IgM, λ, CD20 and CD30 (Ki-1). The primary lesion of lymphoma was suspected to be spleen because of the radiological finding of the spleen. The patient responded to systemic chemotherapy (CHOP) with complete remission. Pathological and clinical characteristics of this B-cell Ki-1 lymphoma were discussed.
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  • Naoko KINUGAWA, Yuri OKIMOTO, Setsuo OTA
    1992 Volume 33 Issue 1 Pages 53-57
    Published: 1992
    Released on J-STAGE: April 24, 2009
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    A 9-year-old boy was admitted with the diagnosis of myelodysplastic syndrome (FAB RAEB in T). The patient was treated with busulfan and cyclophosphamide and transplanted with bone marrow cells from an HLA identical sister. Cyclosporin A (CyA) and short term methotrexate (MTX) was given for prophylaxis against graft verus host disease (GvHD). The serum potassium value was observed to increase to 6.3 mEq/l during the period of CyA therapy. The serum potassium value returned to 4 mEq/l when CyA treatment was decreased to a serum concentration of less than 50 ng/ml (FPIA). On day 90 post transplantation the patient was diagnosed as relapsed. The patient was preconditioned with cyclophosphamide and total body irradiation and a second bone marrow transplantation was performed using cells from the same donor. He was treated again with CyA and short term MTX for the prevention of GvHD. Once again the patient became hyperkalemic with 6.8 mEq/l. The serum creatinine level was 0.9 mg/dl, the GFR was 52.1 ml/min, FEK was 7.1%. Pseudohypoaldosteronism or hyporeninemic hypoaldosteronism was suspected. To investigate this possibility a renin/aldosteron stimulation test was performed. We speculate that an idiosyncratic response to CyA resulted in pseudohypoaldosteronism and produced a defect in potassium secretion.
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  • Seika SATO, Tomoko NAGAI, Yu NISHIKIORI, Keishiro INOUE, Toshitaro NAK ...
    1992 Volume 33 Issue 1 Pages 58-62
    Published: 1992
    Released on J-STAGE: April 24, 2009
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    Coexistence of myeloma and MDS was noted in a patient without history of exposure to cytotoxic drugs. A 73-year-old man was admitted because of fever and dyspnea on exertion. A complete blood count revealed macrocytic anemia with hemoglobin 7.1g/dl, RBC 191×104l and MCV 111.2 fl. WBC was 6,000/μl, with normal differentials. Bone marrow showed erythroid hyperplasia with M/E ratio of 1.36. There were marked tri-lineage cellular abnormalities, which included megaloblastic changes, multinucleated erythroblasts, hypersegmentation of neutrophils, giant neutrophils, and giant platelets. Ringed-sideroblasts were demonstrated in 20% of the erythroblasts. These findings were compatible with MDS. Although plasma cells accounted for only 9.7% of the nucleated marrow cells, there were many immature plasma cells with inclusion bodies, and the patient showed lambda-ight chain type monoclonal gammopathy with corresponding Bence Jones protein. Immuno-histochemical staining of the bone marrow biopsy specimen revealed monoclonal growth of lambda-positive plasma cells. A punched-out lesion of the skull eventually developed. These findings suggest existence of myeloma. There have been some reports of coexistence of MDS and myeloma; supporting the idea of pluripotent stem cell origin of the disease. This is the first documentation of such a case in Japan.
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  • Masahito IWAHASHI, Kimiharu UOZUMI, Hiroichiro UEDA, Shigemi SHIMOTAKA ...
    1992 Volume 33 Issue 1 Pages 63-68
    Published: 1992
    Released on J-STAGE: April 24, 2009
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    A 64 year-old man was admitted to our hospital complaining of fever and edema in Feburuary, 1990. Lymph node biopsy revealed diffuse lymphoma pleomorphic type according to the LSG classification. On hematological examination, leukocyte count was 23,500/μl, of which 36% abnormal lymphocytes expressing CD2, CD3, CD4 and CD25 as same as the lymph node cells. Anti-HTLV-I antibody in serum was positive. From these data, the diaynosis of adult T-cell leukemia (ATL) was made. ATL cells in the blood and lymph node expressed CD30 (Ki-1). CD30 positive ATL cells derived from the blood was increased after short-term culture. The induction of Ki-1 antigen in cell lines and short-term cultured cells from ATL patients was accompanied by the appearence of the HTLV-I related antigen. Then, we suggest that there was some relation between expression of the Ki-1 antigen and activation by HTLV-I in ATL cells.
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  • Takatoshi KOYAMA, Masahide NOGUCHI, Yasuo MARUYAMA, Hideki AKIYAMA, Hi ...
    1992 Volume 33 Issue 1 Pages 69-74
    Published: 1992
    Released on J-STAGE: April 24, 2009
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    A 22-year-old man with T cell type acute lymphoblastic leukemia in first remission underwent autologous bone marrow transplantation (BMT). The preparative regimen included cytosine arabinoside, cyclophosphamide and fractionated total body irradiation. His harvested bone marrow cells were purged with 4-hydroperoxycyclophosphamide. His serological test was positive for cytomegalovirus (CMV) before the BMT. On day 53 after the BMT, he developed dry cough and his chest X-ray film showed bilateral basilar infiltration. Bronchoalveolar lavage was performed and cytology of the specimen revealed typical cytomegaloviral inclusion bodies. DNA analysis and viral culture of the specimen were also positive for CMV. The patient was started on ganciclovir and immunoglobulin with high dose methylprednisolone. His respiratory status deteriorated, however, and the patient expired because of respiratory failure. Autopsy revealed severe interstitial pneumonitis with suppression of CMV replication from the treatment. Interstitial pneumonitis due to CMV should be considered as a significant complication of autologous BMT.
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  • Naoki MORI, Ryosuke OGAWA, Atsushi WAKE, Kazuhiko NAGATA, Tadatsugu SA ...
    1992 Volume 33 Issue 1 Pages 75-81
    Published: 1992
    Released on J-STAGE: April 24, 2009
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    A 60-year-old man born in Miyazaki prefecture was admitted to our hospital complaining of skin rash in December 1989. On hematological examinations, leukocyte count was 14,200/μl with 49% of abnormal lymphocytes showing lobulated nuclei. The surface marker study revealed their phenotype as CD4+8-. Anti human T cell leukemia virus type I (HTLV-I) antibody and monoclonal integration of proviral DNA were positive. From the above results, he was diagnosed as adult T-cell leukemia (ATL). Abnormal lymphocytes gradually decreased without treatment after the first admission. In January, 1990, he began to complain of neck pain. Two months later he was readmitted because of paresis of extremities and disturbance of urination. Vertebral bone mass and a compressed spinal cord in the 4th cervic level were confirmed by MR imaging. He received a resection of tumor and an anterior fusion of vertebrae. The bone tumor was histologically diagnosed as malignant lymphoma, diffuse medium-sized cell type and the infiltrating cells had their phenotype as CD4+8+. He was postoperatively treated with combination chemotherapies, but neurological abnormalities did not improve. He died of pneumonia on 35 days after the operation. A postmortem examination revealed extradural tumor formation with ATL cells. This case is considered to be rare in respect of both the disappearance of most peripheral abnormal lymphocytes without any treatments and the cervical bone tumor showing immunophenotypic change.
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  • Tohru MURAYAMA, Mitsuhiro ITO, Shion IMOTO, Tetsuya TAKAHASHI, Sachiko ...
    1992 Volume 33 Issue 1 Pages 82-86
    Published: 1992
    Released on J-STAGE: April 24, 2009
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    A 59-year-old female with acute promyelocytic leukemia in remission was admitted to our center because of an episode of incidental high fever with general fatigue. She was found to have hepatomegaly. Abdominal CT revealed multiple liver abscesses and a positive culture was obtained for candida albicans from an aspirated abscess. She was treated with percutaneous transhepatic intraportal administration of amphotericin B in additon to oral and intravenous administration. We confirmed the remission of these abscesses by means of the fungal index which is the difference between the values of the limulus test and endotoxin specific test. The fungal index appears to be useful for early diagnosis and treatment of fungal infection.
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  • Akira ISHIGURO, Tatsutoshi NAKAHATA, Yoshiro AMANO, Kenichi KOIKE, Ats ...
    1992 Volume 33 Issue 1 Pages 87-92
    Published: 1992
    Released on J-STAGE: April 24, 2009
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    The cause of aplastic anemia associated with hepatitis (AAH) is as yet still unknown. There is a supposed relation to the immune mechanisms, however few reports have shown the effects of monocytes on the pathogenesis in the patients with AAH. We have reported a case of a 12-year-old boy with AAH related to cytomegaloviruses, and studied the hemopoietic progenitors. He showed pancytopenia and hypoplasia of the bone marrow on admission to our hospital. The culture studies showed that mononuclear cells (MNC) of the bone marrow produced few hemopoietic colonies in all cell lineages. However, the depletion of adherent cells from the MNC increased numbers of erythroid, neutropilmacrophage and megakaryocyte colonies. Furthermore, the additon of adherent cells of the peripheral blood suppressed the colony formation in the aforementioned cell lineages by marrow MNC from which adherent cells, phagocytic cells and T-cells were abrogated. The results way suggest that monocytes play some soles in the pathogenesis of aplasia through inhibitior of hemopoiesis.
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