Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
Volume 29, Issue 1
Displaying 1-17 of 17 articles from this issue
  • —Change of the System in DIC—
    Hayato SHIMADA, Tomoko MICHIMOTO, Midori SUWA, Masanori IKEUCHI, Yoshi ...
    1988 Volume 29 Issue 1 Pages 1-7
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    Physiological changes of coagulation-fibrinolytic system (PT, APTT, Factors I, II, V, VII, VIII, IX, X, XI, XII, SFMC, FDP, tPA antigen, PA activity) during pregnancy, delivery and puerperium, were investigated in 43 healthy non-pregnant (control) and 143 pregnant women. Because of incresed SFMC and coagulation factors (I, II, VII, VIII, IX, X, XII) during pregnancy, pregnant plasma seemed to be hypercoagulable. PA activity did not increase until the first stage of labor (9.1±2.1 IU/ml), while tPA antigen had already increased in the third trimester and reached its peak value (17.7±6.4 ng/ml) immediately after delivery. These results suggest that there is discrepancy between the level of tPA antigen and PA activity during late pregnancy.
    Based on these physiological changes, change of coagulation and fibrinolytic system (plg, α2PI, AT-III, crosslinked FDP, ESR, Plt added to the above factors) were determined in a case of DIC that occurred after abruptio placentae. In this case, stimulation of extrinsic pathway seemed to cause the DIC because of diminished extrinsic coagulation factors. Moreover surprising finding in the present DIC study was that in spite of increased level of tPA antigen (16.4 ng/ml) in peripheral blood plasma, PA activity was significantly decreased (1.8 IU/ml).
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  • Atsuo MARUTA, Hiroko MIYASHITA, Fumio KODAMA, Hiroshi HARANO, Heiwa KA ...
    1988 Volume 29 Issue 1 Pages 8-13
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    Eleven patients with ANLL, two with RAEB and two with hypoplastic leukemia were treated with a low-dose of Ara-C (LDAC) at a dose of 5.9-10.0 mg/m2, s.c. every 12h or 6h. for 14-47 days. Complete remission was obtained in eight patients (53%). CR was obtained in three of five patients with ANLL who had shown no response to the conventional combined chemotherapy of BH-AC DMP or BH-AC AMP. Two patients with overt ANLL occuring from MDS did not respond to LDAC. In the patients with RAEB, CR was obtained in one and only PR in another. CR was also obtained in both patients with hypoplastic leukemia. Severe myelosuppression was observed in all but one patient when one course of LDAC was administered to the patients as a consolidation therapy after CR was once obtained. Relapse was occured in all of the patients. The remission duration was 17-38 weeks (median 25 weeks). However, CR was successfully reinduced with the same LDAC in three of the six patients with relapse. One of them has been tried to submit to a monthly therapy of 10 days of LDAC as a maintenance therapy and has been still in remission for more than 52 weeks after reinduced to CR. These observations suggest that long CR duration cannot be obtained with the LDAC induction therapy alone, though LDAC is of value in the treatment of ANLL.
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  • Takashi HANADA, Hideshi YAMAMURA, Takao EHARA, Satoko NAKAHARA, Akira ...
    1988 Volume 29 Issue 1 Pages 14-18
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    Seven patients with aplastic anemia were successfully treated with immunosupressive agents and peripheral blood T cells were cryopreserved before and after the therapy. Inhibitory activity of cryopreserved T cells to autologous CFU-E was examined after hematological remission. Inhibitory activity was detected in 4 patients before commencing the therapy and it disappeared after remission. Hematopoietic recovery of patients with inhibitory T cells occurred soon after initiation of the therapy compared with that of patients without inhibitory T cells. These findings suggest that inhibitory T cells were closely correlated with the pathogenesis and pathophysiology of aplastic anemia.
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  • Nobuhiro SUZUKI, Takeshi NAGAO, Yutaka NAKAHORI
    1988 Volume 29 Issue 1 Pages 19-26
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    We analyzed DNA samples of Japanese individuals to see whether four intragenic and extragenic RFLPs (restriction fragment length polymorphisms) were useful for carrier detection and prenatal diagnosis of hemophilia A in affected Japanese families, since DNA polymorphisms are sometimes different among ethnic groups.
    Frequencies of heterozygotes in the Japanese females population were 38.3%, 17.4%, 27.9% and 75% for exon14-26/Bcl I, exon26/Bgl I, DX13/Bgl II and St14/Taq I RELPs, respectively. In application for carrier detection of hemophilia A, the intragenic RFLPs were informative in 5 out of 14 hemophilia families (35.7%), and the extragenic RFLPs were informative in 11 out of 14 hemophilia A families (78.6%). When combined, four intragenic and extragenic RFLPs were informative in 11 out of 14 hemophilia A families (78.6%).
    No recombination was observed between the St14 locus and the factor VIII gene, and also between the DX13 locus and the factor VIII gene, but a recombination between the St14 locus and the DX13 locus was observed in one case.
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  • Kazumi SANPI, Yasuhiko KANEKO, Nobuo MASEKI, Masaharu SAKURAI, Masao H ...
    1988 Volume 29 Issue 1 Pages 27-31
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    Twenty-one evaluable patients (30 years of age and younger) with acute non-lymphocytic leukemia were evaluated for remission rate and survival between November 1975 and April 1985 in Saitama Cancer Center.
    Induction therapy was conducted by 7-day course of Daunorubicin (DNR)+Cytosine arabinoside (Ara-C) or Aclarubicin (ACR)+Behenoyl Ara-C (BH-AC). After the induction therapy one course of consolidation therapy was given with the same drugs as these of the induction therapy. After the consolidation therapy, patients were placed on re-inforcement therapy of 5-day course consisting of DNR+Ara-C or ACR+BH-AC given every 5 weeks for 2 years and then every 8 weeks for one more year.
    The overall complete remission rate was 71%. The median survival of all patients was 19 months. Actuarial survival at 5 years was 33%, and was longer than that (16%) for patients more than 30 years of age, although the difference was not statistically significant. The median duration of remission was 40 months, and the curve of remission duration became flat at 4 years with an actuarial relapse-free survival of 38%.
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  • Masaru NAKANO, Saburho KUWABARA, Yoshikazu KAWANISHI, Mami SHIOTA, Yuk ...
    1988 Volume 29 Issue 1 Pages 32-38
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    We reported two cases of B cell lymphoma (BcL) with marked polyclonal hypergammaglobulinemia (PHG) and several autoantibodies (antinuclear antibody, anti-DNA antibody, rheumatic factor and positive Coombs' test etc), but lacking the clinical pictures as autoimmune disease (AID). These two cases were clinically and immunologically compared with six cases with BcL (2 cases; diffuse type and 4 cases; follicular type) having not PHG but a few autoantibodies.
    In the 2 cases with PHG, the elevation of anti-VCA-IgM and anti-EA-DR-IgG titers in addition to the pattern of the past infection (anti-VCA-IgG and anti-EBNA-IgG positive) suggested the reactivation from the latent infection of Epstein-Barr virus (EBV).
    On the other hand, only one kind of autoantibody was detected in each 4 of 6 cases (66%) without PHG and all of them showed the past infection pattern in respect of anti-EBV antibodies.
    From the above-mentioned, the possibility is strongly suggested that the reactivation of EBV is responsible for the immunological events as polyclonal immunoglobulines and autoantibodies production by B cells, when BcL is accompanied by PHG and many autoantibodies, although it is undeniable that the hyperimmune states like AID etc might subclinically precede BcL as the prelymphoma conditions.
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  • Michifumi KOHNO, Makoto MIYATA, Ryuji MATSUYAMA
    1988 Volume 29 Issue 1 Pages 39-43
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    A 52-year-old male born in Hokkaido was admitted to Sapporo City General Hospital because of fever, bleeding tendency and general malaise on May 26, 1986. Hematological examination revealed pancytopenia. The leukocyte count in peripheral blood was 1,100/μl, with 25% abnormal lymphocytes showing convoluted or lobulated nuclei. Serum anti-ATLA antibody was positive with titer of 1:40 (IF) and 1:1024 (PA). The expression of ATLA by short-term culture of leukemic cells was detected. Leukemic cells had T cell markers (Leu 2a(-), Leu 3a(+), Leu 4(+)). Serum immunoglobulin concentration was marked reduced (Ig G 502mg/dl, Ig A 37.9mg/dl, Ig M 73.0mg/dl).
    On the 26th hospital day, he was died owing to the massive melena with small intestine involvement. Postmortem examination showed extensive infiltration of leukemic cells in small intestine, generalized lymphnodes, liver and spleen.
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  • Yoshikazu ABE, Masanori UMEDA, Takaaki KOSUGE, Nozomi ARAI, Tatsuo SHI ...
    1988 Volume 29 Issue 1 Pages 44-47
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    A 59-year-old man was admitted to our hospital because of swelling of the cervical lymph nodes in December, 1985. On admission, his white blood cell count was 18,600/μl, consisting of 20.5% blast cells, 20% monocytoid cells, 18% eosinophils and 15.5% basophils. A bone marrow study revealed hypercellularity with 67.2% blast cells, 1.6% monocytoid cells and 22% eosinophils. Cytochemically, leukemic cells were myeloperoxidase positive. Esterase stainings supported the diagnosis of acute myelomonocytic leukemia. Eosinophils were chloracetate esterase positive. Chromosome analysis revealed 46, XX, -17, +i (17q). Induction chemotherapy with BHAC-DMP and BHAC-AMP regimens resulted in partial remission.
    The number of bone marrow leukemic-colony forming units was 477.5/2 x 105 cells, with 21% monocyte colonies, 24% eosinophil colonies and 10% basophil colonies. The remaining colonies contained various combinations of these three lineages. This suggests that the leukemic clone of this patient arose from a hemopoietic stem cell, for these cells have the capability to differentiate into monocytes, eosinophils and basophils.
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  • Haruo NIIKURA, Yoshio TAKIZAWA, Sadamu OKADA, Shoji HAGIWARA, Hiraku M ...
    1988 Volume 29 Issue 1 Pages 48-53
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    A case of acute myelomonocytic leukemia (AMMoL) associated with a 7: 11 translocation is reported. A 27-year-old Japanese man was hospitalized because of fever, general malaise and leucocytosis (22,700/μl) with atypical blasts and monocytoid cells. A diagnosis of AMMoL was made. BH-AC·DMP therapy was started and it was switched to BH-AC·triple V therapy later, which resulted in complete remission 5 months after initiating chemotherapy. Cytogenetic study of bone marrow cells carried out at the time of admission revealed translocation, t(7;11)(p15;p15) in all of 24 metaphasic cells analyzed by G-banding method. At the time of complete remission the karyotype was normal.
    Ten cases of 7; 11 translocation including the present case have been reported so far. Eight cases are Japanese and two are Caucasian. This abnormality is not necessarily specific to one type of leukemia although it has not been found in lymphoproliferative disorders such as acute lymphatic leukemia. Among the reported cases, the common breakpoint of the short arm of No. 11 chromosome is in p 15 in which the oncogene c-Ha-ras-1 has been mapped. The oncogene c-erb B is supposed to be located in p 14-p 12 of No. 7 chromosome. It would be of importance to clarify whether these oncogenes are involved or not in the leukemic process of 7; 11 translocation-associated myeloproliferative syndromes.
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  • Haruhiko NINOMIYA, Junji TOMIYAMA, Shigehiko IMAGAWA, Akira SHIBUYA, T ...
    1988 Volume 29 Issue 1 Pages 54-58
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    Recurrent thromboses and infections were observed in a 61-year-old man with paroxysmal nocturnal hemoglobinuria (PNH). He complained of abdominal colicky pain and bloody stool. Thromboses of mesenchymal arteries were strongly suspected. He has been also suffering from furuncles of the skin. The granulocytes revealed delayed response against Zymosan and the platelets did not aggregate with epinephrine. ADP and ATP content of the patient's platelets were decreased at the time of abdominal painful attack.
    PNH has been thought to be a clonal disorder of multi-potential stem cell. In addition to its abnormal erythrocytes, granulocytes and platelets also have abnormalities in their functions and their complement sensitivity. We demonstrated decay-accelerating factor (DAF) were deficient on the membranes of his erythrocytes, granulocytes and platelets by FACS analysis and by enzyme-linked immunosorbent assay (ELISA) using monoclonal anti-DAF antibodies. DAF was able to be incorporated to his erythrocytes and the sensitivity against complement lysis was considerablely corrected. It is thought that the deficiency of DAF on blood cells has a profound significance in the presentation of complement-sensitive blood cells.
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  • Kazuma OHYASHIKI, Katsuhiko JUJO, Michi KONDO, Kiyoshi OKADA, Arikuni ...
    1988 Volume 29 Issue 1 Pages 59-63
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    The patient was a 39-year-old male who was diagnosed as having AMMoL (FAB-M4). Chromosome analysis of the leukemia cells on admission revealed a karyotype of 47, XY, +22, inv (16) (p13q22). The patient entered complete remission, however, he relapsed with an orbital tumor and central nervous system (CNS) leukemia. We discuss an involvement of CNS in acute myeloid leukemia with inv (16) (p13q22).
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  • Isao MAEKAWA, Tsugumichi KAWAMURA, Takayoshi MIYAKA
    1988 Volume 29 Issue 1 Pages 64-67
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    A case of chronic adult T-cell luekemia (ATL) complicating disseminated strongyloidiasis was reported. A 64 year-old woman born in Nagasaki prefecture, was admitted in our hospital because of arthralgia and skin rash in April, 1984. The luekocytes counts were 52,100/cmm with 75% of abnormal lymphocytes, their surface marker showed OKT3; 3.6%, OKT4; 98.4%, OKT8; 0.8%. Anti-ATLA antibodies was positive. Serum LDH elevated but no hypercalcemia and no hepatosplenomegaly were showed. The diagnosis of chronic ATL was made. One year after diagnosis, clinical symptoms and hematoligical findings were gradually progressive. Treatments of α-Interferon, total body irradiation and lymphaphersis were done. She complained of dyspnea in April, 1986. The findings of interstitial-pneumonitis revealed in chest X-ray, and ATL cell-infiltration was difined by TBLB specimen. Progression of skin rash and lymphadenopathies were observed, the diagnosis of acute crisis of chronic ATL was made. The treatment of VEPA was slightly effective. Bloody stool appeared in 9 June, 1986. Strongyloides were positive in stool and sputum. Mintezol therapy poorly responded. She died 16th days after the diagnosis of disseminated strongyloidiasis. The causes of death were suspected septic shock and DIC.
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  • Yuzuru KUBO, Hitoshi HIRANO, Masahide ASHIYA, Yasushi SATO, Tetsuo YAM ...
    1988 Volume 29 Issue 1 Pages 68-73
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    Two siblings exhibiting a histiocytic proliferation in the liver, lymph nodes, skin and bone marrow were presented. The cytological features of the proliferating cells were benign-appeared, characterized by uniform contour, abundant eosinophilic cytoplasm and a relatively small round nucleus with a single nucleoli and finely dispersed chromatin. They showed scanty mitotic figures. Active viral infection was not documented by serological tests or viral culture of the lymph node at the time of diagnosis. We considered that the histiocytic proliferation of the present cases was neoplastic rather than reactive, because the clinical course was rapidly progressive and severe and the patients did not respond to the supporting therapy. Furthermore. the treatment of the older brother with aggressive antineoplastic chemotherapies (VEPA, IM) was successful in inducing and maintaining remission for over thirty months. Although the younger brother responded to the same chemotherapies, no complete remission was obtained.
    Since it is very difficult to distinguish morphologically between reactive and malignant nature of the disease, we believe that it might be necessary to manage these patients based on clinical behavior rather than morphology of the histiocytes in the tissues.
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  • Toshiaki SAI, Atushi HOSHINO, Katsuhide HIWATASHI, Masayuki OKA, Tomok ...
    1988 Volume 29 Issue 1 Pages 74-79
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    A 41-year-old male was admitted to Iwaki Kyoritsu General Hospital because of right cranial nerves (VII, IX, X, XI, XII) palsy caused by right suboccipital tumor. The white cell count in the peripheral blood was 7,100/μl containing 12% blast cells. NAP rate was 83% and score was 216. Chromosomal analysis revealed Ph1 abnormality. Using various monoclonal antibodies, the blast cells in the peripheral blood were determined as megakaryoblasts, positive for CDw 41: P2, CDw 41: J 15, CDw 42: AN 51, Plt-1 and KOR-P77. Histological examination of bone marrow revealed megakaryocytic proliferation and moderate replacement by fibrosis. Based on these findings, he was diagnosed as chronic myelogenous leukemia with megakaryocytic transformation without preceding chronic phase. He was treated with irradiation and chemotherapy, but it was difficult to get a complete remission. Seven months after admission, CD11b: MO1, CD13: MY7 and CD14: MY4 positive leukemic cells abruptly increased in the peripheral blood and he died of cerebral bleeding.
    This is probably the first case of chronic myelogenous leukemia with megakaryocytic transformation “without chronic phase”.
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  • Masao TANAKA, Hirokazu NAGAI, Takuhei MURASE, Yoshihisa FUJIWARA, Yasu ...
    1988 Volume 29 Issue 1 Pages 80-84
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    A female patient with primary acqired sideroblastic anemia who terminated in leukemic transformation is described. Hematological studies at the time of diagnosis of sideroblastic anemia revealed marked anemia and poikilocytosis in the peripheral blood and a low percentage of ringed sideroblasts with erythroid hyperplasia in the marrow. Ineffective erythropoiesis was confirmed on ferrokinetics. A marked increase of blast cells was found 3 years later. The patient was given the treatment of low dose Ara-C for 2 weeks, which suppressed the proliferation of blast cells in the marrow. Several courses of low dose Ara-C, each lasting for 20—30 days, were given with an interval of 30—40 days. With each course of treatment, the blasts in the amrrow and the peripheral blood decreased and hepatosplenomegaly reduced. She lived for 20 months after the blastic transformation.
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  • Keisuke TOYAMA, Tetsuzou TAUCHI, Hiromi FUJIEDA, Makoto YAGUCHI, Masat ...
    1988 Volume 29 Issue 1 Pages 85-90
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    A case of aplastic anemia with a 17 years' history which responded to antilymphocyte globulin treatment was reported. The patient was a 54-year-old male who had been followed up under diagnosis of aplastic anemia with prednisolone, oxymetholone, and subsequant mepitiostane treatment until 1986, when he became severely anemic. In January 1987, although he showed rather atypical findings for aplastic anemia such as reticulocytosis and erythroblastosis in the peripheral blood, he received antilymphocyte globulin (Merieux) and responded well to this treatment. Neither colony formation of T-depleted bone marrow cells nor detection of 20.3 (monoclonal antibody)-positive cells in the peripheral blood by fluorocytometry could be an indicator predictive of response to ALG therapy. It may be suggested that antilymphocyte globulin therapy would be recommended for the treatment of even atypical aplastic anemia.
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  • Hiroshi FUJII, Taira MAEKAWA
    1988 Volume 29 Issue 1 Pages 91-97
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    Three patients with thrombotic thrombocytopenic purpura (TTP), diagnosed on the basis of thrombocytopenia, microangiopathic hemolytic anemia, fever, neurological manifestations and renal insufficiency, were treated with plasma exchange (PE). Using a membrane plasma separator (AP-05, Asahi Medical Co.), exchanges of 2-3 liters were performed. Replacement was made with an equal volume of fresh frozen plasma. All patients were treated with antiplatelet agents and corticosteroid, as well as PE.
    Patient 1 showed a transient neurological improvement. But hematological remission could not be obtained and she died on the 110th hospital day. Patient 2 had a dramatic complete response during a short term. But a relapse occurred twice and a complete response was obtained with each PE. Thereafter he received splenectomy and then maintained a complete remission. Patient 3 relapsed after an initial response and treated with vincristine combined with PE and antiplatelet agents. She showed a transient hematological improvement, but died on the 82nd hospital day. Recently the combination therapy with PE and antiplatelet agents is regarded as the first choice for treatment of TTP. However, patients who fail to achieve a complete remission after this therapy should be immediately treated with the salvage therapy, e. g. splenectomy, vincristine and so on.
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