Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
Volume 31, Issue 5
Displaying 1-26 of 26 articles from this issue
  • Hisamitsu SUZUKI, Atsushi OYAMA
    1990 Volume 31 Issue 5 Pages 534-540
    Published: 1990
    Released on J-STAGE: March 12, 2009
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    Consecutive 431 patients with B-cell lymphoma seen in Aichi Cancer Center Hospital from 1964 to 1988 were analysed. Median age was 56 yr (range 8-86). There were 76 patients (17.6%) with follicular lymphoma and 355 with diffuse lymphoma. Among the 30 patients with follicular lymphoma, 10 (33%) were found to have a bcl-2 rearrangement. The incidence was lower than those reported in the United States. This might contribute to the lower incidence of follicular lymphoma cases in Japan. There were 168 patients (39.0%) with nodal lymphoma and 263 with extranodal lymphoma. Eighty-four cases arose from the stomach, 84 from Waldeyer's ring, 21 small intestine and 13 thyroid. The distributions of extranodal B-cell lymphoma differed from those of T-cell lymphoma. Patients with gastric lymphomas in stage I were treated with resection alone. In the same manner, patients with Waldeyer's ring lymphoma in stage I were treated with radiotherapy alone. More than 90% of these patients were expected to survive without relapse. On the other hand, only 55% of patients with nodal lymphoma in stage I were expected to survive for more than 10 years. These findings suggest the site of localized lymphoma is an important determinant of outcome of clinical behavier.
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  • Ryuichi AMAKAWA, Fumio MATSUYAMA, Shirou FUKUHARA
    1990 Volume 31 Issue 5 Pages 541-546
    Published: 1990
    Released on J-STAGE: March 12, 2009
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    Using three chromosome 18-specific DNAs, rearrangements of a bcl-2 gene were detected in 20 (44%) of 45 Japanese patients (pts) with follicular lymphoma (FL) and 3 (8%) of 36 pts with diffuse large cell lymphoma. The 21 pts had t(IgH; bcl-2), and of the remaining two who did not display it, one had chromosome t(14;18). Compared with the findings in American pts, the lower frequency of t(14;18)-positive lymphoma could reflect a difference in the incidence of overall nodal B lymphoma between Japan and the United States. 11 of 18 pts with t(14;18)-positive FL expressed CD10 antigen on the cell surface and all 12 pts with t(14;18)-negative FL did not, and the difference is statistically significant, indicating that t(14;18)-positive Japanese FL is the same disease entity as most of American FL. Extra 18q- chromosome found in the advanced grade diseases of t(14;18)-positive lymphoma results in amplification of the rearranged bcl-2 gene on 18q-, suggesting that the change is closely associated with transformation of FL carrying t(14;18). It is thus possible of international realization to institute the prognostic factors and the treatment strategy for conquerring t(14;18)-positive lymphoma.
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  • Katsuyuki AOZASA, Masahiko OHSAWA
    1990 Volume 31 Issue 5 Pages 547-553
    Published: 1990
    Released on J-STAGE: March 12, 2009
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    Our previous study suggested a close relationship between a preceding chronic tuberculous pyothorax and the development of non-Hodgkin's lymphoma (NHL) in the pleural cavity. To confirm this further, 37 cases were collected, their clinical and pathological findings summarized. The age at first admission for lymphoma of patients ranged from 46 to 81 (mean 63) years, the male to female ratio being 5.2:1. All patients were admitted after a 22-55 (mean 33) year history of pyothorax resulting from artificial pneumothorax for treatment of pulmonary tuberculosis (29 cases) or tuberculous pleuritis (seven cases). The most common presenting symptom was chest pain. The diagnosis of pleural NHL was made by biopsy for 31 of the patients and at autopsy for the other six. Histologically 30 (81%) of 37 cases were of diffuse large cell type, and of these the immunoblastic type was the most common (22 cases). Immunological and immunohistologic studies revealed a B-cell nature of the proliferating cells in all but one tumor. Thirty-two patients received chemotherpy and/or radiotherapy. Twenty-seven patients died between one and 144 (median eight) months of diagnosis. Autopsies carried out in 23 cases revealed the disease to have been localized to the thorax in 11 patients. These findings indicated that malignant B-cell lymphoma arose as a monoclonal growth from a pool of proliferating polyclonal B lymphocytes in tissues affected by the chronic tuberculous pyothorax.
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  • Tohru KOBAYASHI, Kenkichi KITA, Toshiyuki OHNO, Shigeru SHIRAKAWA
    1990 Volume 31 Issue 5 Pages 554-563
    Published: 1990
    Released on J-STAGE: March 12, 2009
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    Forty-one patients diagnosed as having B-cell chronic lymphocytic leukemia in Japan were evaluated. Patients were classified into five groups (CLL, CLL-L, CLL/PL, PLL, LSCL) according to differential counts of small lymphocytes, large lymphocytes, prolymphocytes, immunoblasts and/or lymphosarcoma cells among the lymphoid cells in the first available peripheral smears based on Melo's morphological criteria. Patients with typical CLL, difined as having more that 90% small lymphocytes, were seven (17%) of 41 patients.
    Phenotypical characteristic of CLL and its subgroups in Japan was considered to be the high expression of activated antigens, such as CD22, PCA1 and CD25. This finding may support the relatively higher proportion of atypical CLL with high percentage of large lymphocytes or prolymphocytes in Japan than that in Western countries. The molecular biological analysis revealed the deletion of Cμ gene in one allele in three sIg-negative cases. The incidence of CLL, CLL-L and CLL/PL in Japan was deduced to be 1/10 of that in Western countries.
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  • Kensei TOBINAI, Masanori SHIMOYAMA
    1990 Volume 31 Issue 5 Pages 564-568
    Published: 1990
    Released on J-STAGE: March 12, 2009
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    Immunophenotypic anaysis on 34 cases of T-cell malignancies using monoclonal antibodies against T-cell receptors (TCR) revealed 25 cases of αβ-type and two of γδ-type. The two patients with γδ-type showed cutaneous involvement of tumor cells.
    Immunoblastic lymphadenopathy (IBL)-like T-cell lymphoma is divided into three histologic categories; inconspicuous type, patchy type and diffuse type. DNA hybridization analysis revealed that 11 of 16 cases showed clonal rearrangement of TCR β-chain gene without rearrangement of immunoglobulin heavy chain gene, providing strong evidence for clonal proliferation of T-cells.
    Among 185 patients with adult T-cell leukemia (ATL), 18 cases (9.7%) were found not to be associated with human T-cell leukemia virus type I (HTLV-I). They consisted of 10 of acute type, five of chronic type, two of lymphoma type and one of smoldering type, indicating a diversity in clinical features.
    Two Japanese patients with ATL developed secondary monoclonal B-cell lymphomas of diffuse, large cell, non-Burkitt type. They were seropositive for HTLV-I but negative for human immunodeficiency virus (HIV). They also suffered from pulmonary tuberculosis, and one from adenovirus type 11-induced hemorrhagic cystitis, indicating an immunodeficient state. Epstein-Barr virus genome was found in lymphoma cells from one patient. It is suggested that opportunistic B-cell lymphomas may occur in the immunodeficient stage of ATL.
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  • Takashi OKAMOTO
    1990 Volume 31 Issue 5 Pages 569-571
    Published: 1990
    Released on J-STAGE: March 12, 2009
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    A multi-step model of the carcinogenesis for adult T-cell leukemia-lymphoma (ATL) has been reviewed here according to the authors observations. Epidemiological studies demonstrated that in most japanese ATL cases the disease developed from those who acquired infection of the causal agent, human T-cell lymphotropic virus type 1 (HTLV-1), during early infancy, probably through breastfeeding. Therefore, the latency period between the acquisition of HTLV-1 and clinical manifestation of ATL can be represented by the age of disease onset. It has been demonstarted by us that the age distribution of ATL onset can be described by a single Weibull distribution function, which is considered to be a feasible mathematical model for multistep carcinogenesis. Based on the present model, it is assumed that age-dependent accumulation of nearly five leukemogenic events, most likely somatic mutations, within the target cell (s) might be required prior to the disease onset.
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  • Saburo MINAMI, Kazuyuki NAITO
    1990 Volume 31 Issue 5 Pages 572-576
    Published: 1990
    Released on J-STAGE: March 12, 2009
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    We analysed probability of disease free survival (DFS) and remaining in remission (POR) in evaluable patients with hematological malignancies undergoing allogeneic bone marrow transplantation by Nagoya BMT groups between 1976 and August 1989 according to conditioning regiemens retrospectively. Patients were divided into good risk patients with acute leukemia in first remission or CML in chronic phase and high risk patients with advanced disease.
    The results are as follows:
    1. DSF and POR in good risk patients was 45% and 68% at 7 1/2 years for thirty nine patients treated by CY+TBI, 63% and 78% at 5 2/3 years for thirty nine patients treated by CA+CY+TBI, 84% and 84% at 2 years for sixteen patients treated by preconditioning regiemens without TBI respectively.
    2. DFS and POR in high risk patients was 21% and 45% at 6 years for twenty nine patients treated by CY+TBI, 23% and 48% at 4 5/6 years for forty patients treated by CA+CY+TBI, 64% and 74% at 2 years for eight patients treated by preconditioning regiemens without TBI respectively.
    3. There were no statistical differences among these conditioning regiemens in good and high risk patients. These results show that more effective and stronger preconditioning regiemens are needed especially for high risk patients for prevention of posttransplant leukemia relapses.
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  • Akira HIRAOKA
    1990 Volume 31 Issue 5 Pages 577-583
    Published: 1990
    Released on J-STAGE: March 12, 2009
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    Considering the merit and demerit of GVHD in leukemia patients following allogeneic BMT, our strategies for GVHD are as follows; (1) a mild prevention, (2) a treatment to control the severity, if occurred, and (3) an induction of GVHD in recipients who did not develop GVHD. We reported the results of recent trials in prevention, treatment and induction of GVHD. For the prevention, T cells were depleted from bone marrow cells in 11 recipients with the results of graft failure in 5 and death in 9. The data from IBMTR were similar to our data. For the treatment, new drugs, 15-Deoxyspergualin, Mizoribine and anti-human lymphocyte globulin were introduced with compromising outcomes and mild adverse effects. For the induction, Ubenimex, an immunostimulator, was administered to recipients who did not develop GVHD until 30 days after BMT. The interim data suggests that Ubenimex may induce chronic GVHD and suppress the relapse of leukemia. We experienced the sudden onset of GVHD during the tapering of immunosuppressants in two recipients, who were found to have arbitrarily discontinued them. The interruption of drugs for the prevention of GVHD may cause the induction of GVHD.
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  • Minoru OTSUKA, Takao MORI
    1990 Volume 31 Issue 5 Pages 584-588
    Published: 1990
    Released on J-STAGE: March 12, 2009
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    Allogeneic bone marrow transplantation (BMT) has been used successfully to treat patients with hematological malignancies. However, interstitial pneumonia has remained a major complication following BMT. We investigated the possibility of immune transfer in recipients treated with BMT from immunized donors. Bone marrow donors were vaccinated with tetanus toxoid (TT) before marrow harvest. After BMT, serum IgG anti-TT antibody titer and in vitro IgG anti-TT antibody production in recipients were assayed sequentially. IgG anti-TT antibody titer in serum increased from about day 20 after BMT. In addition, spontaneous IgG anti-TT antibody producing B cells and TT antigen-specific memory B cells derived from donors were also detected in peripheral blood and bone marow of recipients from about day 60.
    We therefore considered that antigen-specific immunity of bone marrow donors is transferable to recipients with BMT, and the immunity continued for a long time in the recipients after BMT.
    The effectiveness of cytomegalovirus (CMV) vaccine on antibody production was next investigated in healthy adults. Live vaccine induced the production of humoral antibody but envelope vaccine did not. These results indicate that more effective procedure should be needed for the use of envelope vaccine.
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  • Satoshi TAKAHASHI, Hideki KODO
    1990 Volume 31 Issue 5 Pages 589-596
    Published: 1990
    Released on J-STAGE: March 12, 2009
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    Human granulocyte colony-stimulating factor (G-CSF) specifically stimulates granulocyte production and enhances functions of mature granulocytes. It also proliferates myeloid leukemic cells. The molecule was purified and molecularly cloned in 1986. In this study, 51 patients received single daily injections with recombinant human (rh) G-CSF (2-20 μg/kg) after bone marrw transplantation were compared with control 36 patients. Blood neutrophilic recovery was accelerated remarkably by rhG-CSF administration without any adverse effects including delay in reticulocyte and platelet recoveries. Furthermore, the duration for the management in laminar airflow room and febrile days ≥38°C were shortened in rhG-CSF treated group. In 6 patients with myeloid leukemia in relapse, we also tested the effects of the rhG-CSF-combined conditioning regimen. In all the patients tested, the leukemic cells responded to rhG-CSF. Clinically no relapse was observed and 3 patients are well on day 224-357 for the time being. These findings indicate that rhG-CSF is very useful in bone marrow transplantation.
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  • Koichi MIYAMURA, Kuniyuki IMAI, Shuki MIZUTANI
    1990 Volume 31 Issue 5 Pages 597-602
    Published: 1990
    Released on J-STAGE: March 12, 2009
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    Polymerase chain reaction (PCR) is a recently-developed technique capable of amplifying a specific nucleotide sequence and has been proved highly sensitive enough to analyse a small amount of DNA. This prompts us to study minimal residual disease (MRD) which is undetectable by conventional morphological or cytogenetic analysis. Using this technique, tumor specific chimeric DNA or mRNA were detected in patients with follicular lymphoma with t(14;18) or in Ph1 positive ALL in complete remission, respectively. PCR was also useful to monitor MRD after treatment of bone marrow cells with monoclonal antibody. In sex-mismatched bone marrow transplantaion, mixed chimerism was well documented by using repeated sequences which is unique to human Y chromosome. These results suggested that PCR offers a great help for detecting MRD or mixed-chimerism following bone marrow transplantation.
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  • Kosei MATSUE, Kozue NAKAMURA, Shuki MIZUTANI
    1990 Volume 31 Issue 5 Pages 603-608
    Published: 1990
    Released on J-STAGE: March 12, 2009
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    We have ultilized the polymerase chain reaction (PCR) to sensitively detect the minimal residual leukemia in 24 patients who were hematologically normal and were negative for Ph1 chromosome after bone marrow transplantation (BMT). None of the patient showed breakpoint cluster region (BCR) gene rearrangement by Southern blot analysis, however, PCR tequnique revealed the bcr/abl mRNA in 13 of 24 patients. In patients who received CY+TBI as a conditioning regimen, 6 out of 8 patients were detected bcr/abl mRNA by PCR. On the other hand, patients who received high dose AraC+CY+TBI or busulfan+CY as a conditioning, bcr/abl mRNA was detected in 4 out of 9 patients and 1 out of 5 patient, respectively. There was no clear association between the presence or absence of graft versus host disease and the detection of bcr/abl mRNA by PCR.
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  • Yoichi TAKAUE, Tsuneo NINOMIYA
    1990 Volume 31 Issue 5 Pages 609-613
    Published: 1990
    Released on J-STAGE: March 12, 2009
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    This paper reports a clincal and laboratory experience of peripheral blood stem cell (PBSC) autografts at a single institute. Twenty-eight children with various types of cancer underwent a total of 90 leukaphereses to collect PBSC and 17 of them subsequently received marrow-ablative therapy and PBSC autografts. We found that frozen-thawed progenitor dose is important in determining the rate of hematopoietic recovery after transplantation; in 11 patients who received more than 1×105 CFU-GM/kg, the granulocyte count reached to 0.5×109/L in two weeks. With conditioning chemotherapy without total body irradiation, 6 of 14 patients with high-risk acute leukemia or non-Hodgkin's lymphoma have survived disease-free 3 to 25 months posttransplant.
    This approach may have a potential to induce prolonged remission-interval and ultimate cure.
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  • Hiromasa YABE, Shunichi KATO
    1990 Volume 31 Issue 5 Pages 614-619
    Published: 1990
    Released on J-STAGE: March 12, 2009
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    Two patients treated by unrelated bone marrow transplantation were reported.
    Case 1 was an eight-year-old boy with Morquio's disease received bone marrow graft from an HLA one-locus mismatched, MLC non reactive unrelated donor. The patient was prepared with conventional dose of cyclophosphamide, and thoracoabdominal irradiation. For the prophylaxis of GVHD, three drug regimen consisted of methotrexate (MTX), cyclosporine A (CsA), and prednisolone (PSL) was administered. Engraftment was prompt, and grade I of acute GVHD developed, which resolved with increased dose of PSL.
    Case 2 was a ten-month-old boy with juvenile chronic myelogenous leukemia received bone marrow graft from an HLA fully matched, MLC non reactive unrelated donor. Preconditioning regimen consisted of total body irradiation, VP-16, and cytosine arabinoside. MTX, CsA, and methylprednisolone were administered to prevent GVHD, but grade II of acute GVHD developed, which resolved with prolonged course of PSL.
    Both cases are alive and well, without chronic GVHD. In conclusion, unrelated donor bone marrow transplantation may be a useful tool to treat hematologic malignancies, aplastic anemia, and some inherited diseases.
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  • Shinichi MIZUNO, Yasuo MORISHIMA
    1990 Volume 31 Issue 5 Pages 620-624
    Published: 1990
    Released on J-STAGE: March 12, 2009
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    Tokai Bone Marrow Donor Registry was established to facilitate unrelated bone marrow transplantations in Japan. Although the basic structure was constructed, the number of donors must be expanded and financial problems must be solved. More bone marrow donor registries are likely to be established in the near furure throughout Japnan, and those will be anticipated to become a nationwide bone marrow donor registry.
    The HLA identity between unrelated indivisuals is unique in that it is only phenotypically identical and not genotypically identical. Therefore, for the HLA matching, not only serological typing of donors and recipients but molecular and biochemical methods would be employed to facilitate the analysis of the degree of the HLA identity to be more precise. Those methods include HLA-DP DNA typing and IEF analysis of HLA-class 1 antigens.
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  • Koichi SHIMMYOZU, Toshihide OKADOME, Yoshikazu MARUYAMA, Naoko KADOKUR ...
    1990 Volume 31 Issue 5 Pages 633-638
    Published: 1990
    Released on J-STAGE: March 12, 2009
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    Twenty-one patients with anti-phospholipid antibodies (APLA), who were selected from 104 patients with antinuclear antibody or anti-DNA antibody, were studied to define clinical characteristics of APLA.
    Of the 21 patients, the incidences of IgG anti-cardiolipin antibody (ACA), IgM ACA, lupus anticoagulant (LAC) and BFP-STS were 20, 7, 11 and 12, respectively, and they were highly related with each other. The number of items of the 1982 ARA revised criteria for the classification of SLE was significantly low (mean value=3.3), and also the level of serum C4 was significantly high (mean value=88% of normal) compared with those in patients without APLA. The incidence of thrombocytopenia and hemolysis was significantly high when compared with those in patients without APLA, and they were closely related to the presence of LAC. The incidence of thrombosis was markedly high (48%), 10 of 21 patients, especially those of cerebral infarction and deep vein thrombosis. Cerebral infarction was significantly associated with LAC. The obstetric complication was 4 of 13 patients (31%) in the incidence, which was significantly high compared with that in patients without APLA. Of them, natural abortion was closely associated with LAC.
    Based on these observations, it seems that autoimmunological disease activity in patients with APLA may be slight to mild, and strongly suggested that APLA may play an important role particularly in the pathogenesis of acquired thrombotic tendency.
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  • Hitoshi TAKEUCHI, Isao KATAYAMA
    1990 Volume 31 Issue 5 Pages 639-646
    Published: 1990
    Released on J-STAGE: March 12, 2009
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    In 8 cases of myeloid antigen positive (My+) ALL (L2), morphology and phenotype of their blasts and the differentiation-inducing effect of each of 12-o-tetradecanoyl phorbol-13-acetate (TPA) or recombinant GM-CSF (rGM-CSF) on the blasts were analysed.
    Five cases expressed common ALL phenotype such as CD19+, CD20-∼+, CD10+, DR+ and c-μ-∼+, and also My antigens such as CD11c+, CD11b+, CD14+ and CD13+ (B+My+ALL). One case expressed T-ALL phenotype such as CD5+, CD2+, CD3+, CD8+, CD4+ and CD10+ and also My antigen such as CD14 and CD13 (T+My+ALL). These six cases were diagnosed as biphenotypic ALL. The other 2 cases expressed mainly My antigen such as CD11c+, CD11b+, CD14+, CD13+, CD33+ and DR+, and one of the two expressed also CD2 (B-T-∼+My+ALL).
    TPA induced the marked morphological change to monocyte-macrophage like cells and the increase of CD11+ cells in both of B-T-∼+My+ALL cases. rGM-CSF transformed the blasts to large cells or cells with azurephilic granules in B+My+ALL cases.
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  • Kumiko TAMURA, Masaaki KOSAKA, Takashi MIZUGUCHI, Yoshiyuki MIYAMOTO
    1990 Volume 31 Issue 5 Pages 647-654
    Published: 1990
    Released on J-STAGE: March 12, 2009
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    The number of circulating hematopoietic progenitor cells was determined during 47 courses of chemotherapy in 23 patients with hematopoietic malignancies. rhG-CSF was given subcutaneously to 14 patients to rescue chemotherapy-induced neutropenia following 22 courses of chemotherapy. The mean numbers of CFU-GM in patients with malignant lymphoma (ML), acute leukemia (AL) and myeloma (MM) were 56.0±58.8 (mean±SD), 46.7±66.0 and 11.0±11.1 CFU-GM/ml, respectively. The number of CFU-GM in MM was significantly less than in normal subjects (51.2±30.6 CFU-GM/ml). The number of CFU-GM in PB in all patients began to rise between 2 days before and 8 days after nadir of WBC count, and then reached the peak at the subsequent 5 days. The peak values of CFU-GM in ML, AL and MM were 711.3±974.7, 660.0±374.7 and 403.6±232.5 CFU-GM/ml, respectively, but there was no statistical difference among them. When ML patients were treated with rhG-CSF, the CFU-GM peak values increased as much as 5.5-folds compared with those following chemotherapy only. However, neither the period from nadir to start of increase in the CFU-GM count nor the time of the CFU-GM peak showed any significant change. These results indicate that the administration of rhG-CSF makes it possible to increase the number of circulating progenitor cells. It appears possible in most of the patients with hematopoietic malignancies to harvest the sufficient number of progenitor cells which are necessary for autologous blood stem cell transplantation.
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  • —Productive Ability of Colony Stimulating Activities and Interferon-γ by Blood Mononuclear Cells in Patients with Aplastic Anemia—
    Yoshifumi KAWANO
    1990 Volume 31 Issue 5 Pages 655-659
    Published: 1990
    Released on J-STAGE: March 12, 2009
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    To evaluate the role of cytokines in patients with aplastic anemia, colony stimulating activities (CSA) and interferon-γ (IFN-γ) in cultured media of lymphocytes with phytohemagglutinin (PHA-LCM) were measured with methylcellulose culture method in 20 patients (age 3 to 69 years). The CSA for granulocyte/macrophage (GM-CSA) in patients was equivalent to that of normal donors, while low burst promoting activity (BPA) was observed in PHA-LCM from 7 adult patients (61±17%). The ability of BPA production varied widely in 13 children (97±37%). In some patients, low production of BPA improved after successful treatment of antilymphocyte globulin. The IFN-γ in PHA-LCM disclosed no significant difference between patients and normal donors. From these results, low production of BPA may have a role in the development of AA in certain patients. It is also suggested that therapy with recombinant cytokines such as GM-CSF and IL-3, detected as BPA in our culture system, could be effective for those patients.
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  • —Congenital Platelet Membrane Glycoprotein VI Deficiency—
    Koichi SHINMYOZU, Masaru KURIYAMA, Toshihide OKADOME, Yoshikazu MARUYA ...
    1990 Volume 31 Issue 5 Pages 660-664
    Published: 1990
    Released on J-STAGE: March 12, 2009
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    Previously, we reported a case of 26-year-old woman with a mild bleeding tendency whose platelets specifically lacked collagen-induced aggregation and adhesion to collagen fibrils. In this report, we investigated the membrane glycoproteins of this patient's platelets and found that her platelets were absent in a 61-kDa glycoprotein, which was identified to be glycoprotein VI (GP VI) of the platelet membrane. Her parents platelets contained about 50% the normal amount of GP VI. These results indicate that our patient has a congenital homozygous GP VI deficiency and that GP VI functions as a collagen receptor.
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  • Koichi SHINMYOZU, Toshihide OKADOME, Yoshikazu MARUYAMA, Ikuro MARUYAM ...
    1990 Volume 31 Issue 5 Pages 665-668
    Published: 1990
    Released on J-STAGE: March 12, 2009
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    Occurrence of the two congenital hemorrhagic disorders, von Willebrand disease (vWD) and hemophilia A, was found in the same family.
    The propositus was a 21-year-old male patient with findings of moderate hemophilia A. Father, the eldest sister and second eldest sister were found to have mild type I vWD. Mother was confirmed to be a hemophilia A carrier, and the eldest son of second eldest sister to be a moderate hemophilia A. These results suggest that the occurrence of the two hemorrhagic disorders may have been resulted from the incidental mating of father's vWD gene and mother's hemophilia A gene, and suggest that second eldest sister may be the double heterozygosity fo type I vWD and hemophilia A.
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  • —A Case Report—
    Masamichi BABA, Norio TANAHASHI, Kyoichi SENO, Masaharu NARA, Takuro S ...
    1990 Volume 31 Issue 5 Pages 669-673
    Published: 1990
    Released on J-STAGE: March 12, 2009
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    A 22-year old male was admitted to Ashikaga Red Cross Hospital because of the progressing abdominal distension in May 1988.
    The physical examinations disclosed marked hepato-splenomegaly, lymphadenopathy and cachexic state. In the laboratory examinations, anemia, leukocytosis with left shift of nuclei and severe inflammatory reactions were found.
    Bone marrow aspirations were dry taps, and the biopsy showed myelofibrotic changes. Abdominal computed tomography and ultrasonography revealed a marked hepatomegaly and a giant splenomegaly with subcapsular hematoma. Philadelphia chromosome was negative and neutrophilic alkaline phosphatase score was slightly high. A little amount of peritoneal effusions was obtained and yielded S. aureus.
    A diagnosis of myelofibrosis with subcapsular hematoma due to spontaneous rupture of spleen and peritonitis of unknown cause was made. Operative indication was considered, but his family did not agree with it. Then conservative therapies for general conditions, those are, administrations of antibiotics and diuretics, and blood transfusions were taken. On the enlarged spleen, total irradiation was done. After the therapies, his spleen got smaller and hematoma was going to be absorbed.
    A rare case of myelofibrosis with splenic hematoma is reported and the conservative therapies were effective to this case.
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