Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
Volume 29, Issue 11
Displaying 1-50 of 51 articles from this issue
  • Hiromu FUKUI
    1988 Volume 29 Issue 11 Pages 1980-1990
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    Von Willebrand's disease (vWD) is an inherited bleeding disorder due to deficiency or abnormality of von Willebrand factor (vWF), a large multimeric glycoprotein which forms a noncovalent complex with factor VIII in blood. On the basis of the structure-function relationship of F. VIII/vWF complex, the disease can be divided into three main states; type I (classical form), type II (variant form) and type III (severe, homozygous form). A bleeding disorder with an intrinsic platelet abnormality affecting platelet-vWF interaction is called pseudo-vWD or platelet type vWD.
    This paper will review 1) Structure function relationship of vWF; 2) Diagnosis of vWD; 3) Ristocetin induced platelet aggregation and Botrocetin induced platelet aggregation in various types of vWD; 4) Plasma multimeric composition of vWF in various types of vWD; 5) vWF-subunit structure in various types of vWD; and 6) Hemostatic effects of DDAVP and heated factor VIII concentrates.
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  • Makoto AKASHI, Masatsugu OHTA, Seiichi KITAGAWA, Yusuke FURUKAWA, Koui ...
    1988 Volume 29 Issue 11 Pages 1991-1997
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    Fifty previously untreated patients with multiple myeloma were reviewed for a retrospective study of their classification according to renal function (subclassification proposed by Durie and Salmon), and a combination chemotherapy including an alkylating agent “Carboquone” was discussed. In the group with renal failure there were more patients in stage III than in the group without, and the main cause of death was renal failure. The percentage of decrease in serum-creatinine before and after chemotherapy was demonstrated to be proportional to the response rate of chemotherapy. The response rate for the combination of carboquone and prednisolone was 92.3%. These findings suggest that renal function reflects the total amount of body myeloma and that an active removal of renal failure by such as dialysis might to lead to the long survival. In addition. consideration should be given into a new drug combination including carboquone in future clinical trials.
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  • Tetsunori FUNABIKI, Sumio KAI, Keiji FUKAZAWA, Shumpei YOKOTA, Koichir ...
    1988 Volume 29 Issue 11 Pages 1998-2003
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    Anti-platelet antibody in the sera from thirty-one patients with thrombocytopenia, and the platelet antigen which the antibody recognized, were studied by the method of immunoblotting. Anti-platelet antibody were detected in two patients with chronic idiopathic thrombocytopenic purpura (ITP), out of four acute ITP, twenty-two chronic ITP, three aplastic anemia, two acute lymphocytic leukemia that were refractory to platelet transfusion. Platelet antigen that the antibody recognized was apparently different from HLA-ABC antigen, and their molecular weights were 90kD, 55kD, 48kD in one case, 50kD, 38kD, 37kD in the other case. The diversity of the platelet antigen detected in this study indicates the complexity of the pathogenesis of chronic ITP.
    Two antigens, of which molecular weight were 50kD and 38kD, were detected in the mononuclear cell fraction of normal peripheral blood. It is not clear whether these antigens were expressed constitutively on the mononuclear cell, or platelet antigen were adsorbed on the surface of mononuclear cell.
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  • Hirokazu MURAKAMI, Tsutomu YANAGISAWA, Norifumi TUKAMOTO, Hitoshi KURA ...
    1988 Volume 29 Issue 11 Pages 2004-2010
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    Durie et al. classified patients with multiple myeloma into 4 groups (A, B, C, D) according to their pattern of response to chemotherapy. In our study, 87 of 120 patients were classified as pattern A, and there was no significant difference in the median survival duration among the groups; 34 months in A, 31 months in B, 78 months in C, and 43 months in D. Therefore, we classified the patients in pattern A into 4 subgroups; Al: a high M component at diagnosis and low tumor regression rate (<50%), A2: a high M component and high tumor regression rate (50%≥), A3: a low M component and low tumor regression rate (<50%), A4: a low M component and high tumor regression rate (50%≥). There was no significant difference in the number of patients; A1: 12, A2: 27, A3: 23, A4: 25. The median survival duration was 34 months in A1, 22 months in A2, 103 months in A3 and 21 months in A4. The median survival was longer in A1 than in A2, and it was significantly longer in A3 than in A2 or A4 (p<0.05). As above, the tumor regression rate after chemotherapy is an important prognostic factor equal to the M component at diagnosis in patients with multiple myeloma. Additionally, the percentage of myeloma cells in bone marrow at diagnosis are the good indicator for the prediction of response pattern of multiple myeloma to chemotherapy.
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  • Hideo WADA, Mikio TAKAGI, Yoshitaka MORI, Akira DEGUCHI, Hikoji SUZUKI ...
    1988 Volume 29 Issue 11 Pages 2011-2017
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    We performed blood coagulation studies in 50 patients within a week before onset of disseminated intravascular coagulation (DIC).
    Since two peaks were observed for the onset, it was suspected that DIC was composed of acute and chronic types. The DIC score before onset was 4.9±1.2 in the non-leukemic group and 2.4±0.8 in the leukemic group.
    Prekallikrein activity, FDP, fibrinogen and platelet number were frequently abnormal, but PT and antithrombin III weré slightly abnormal in the non-leukemic group.
    Although the incidence of abnormal finding was lower in the leukemia group than in the non-leukemia group, the data on the leukemia group were similar to those on the non-leukemic group and elevated D-dimer level and decreased platelet count were particularly frequent in the leukemic group. The incidence of abnormal data in all patients was 44±14%.
    Abnormalities of blood coagulation were observed within a week before the onset of DIC, and prekallikrein activity, platelet number, FDP and D-dimer might be useful for diagnosis of Pre-DIC state.
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  • Yoshinobu TAKEMOTO, Yoshiki SHINOHARA, Yoshihiro FUJIMORI, Sumire ANDO ...
    1988 Volume 29 Issue 11 Pages 2018-2023
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    We compared the outcome of bone marrow transplantation with that of continued chemotherapy for acute leukemia who achieved a first remission. From August 1981 to December 1986, 141 consecutive patients (ages 9 to 77 years) with acute leukemia were treated with induction chemotherapy. One hundred and four patients (74%) entered a complete remission. Of those, 42 patients were under the age of forty years. Sixteen remission patients had HLA-matched siblings. Eleven of them received bone marrow transplantation (BMT group) and five did not (BMT refusal group). Twenty six patients in remission without matched siblings were treated with continued chemotherapy (chemotherapy group).
    Kaplan-Meier estimates of 4-year disease free survival from complete remission were 73% for the transplanted group and 14% for the chemotherapy group. Within the transplant group, three partients died during the first six months and eight patients were alive in complete remission. All of the BMT refusal group relapsed within eight months. In the chemotherapy group, 22 of 26 patients relapsed and/or died during 16 months.
    Among the transplant group, ages under 25 years and the presence of acute GVHD grade I were associated with better prognosis. In the continued chemotherapy group, high initial leukocyte counts were related to poor prognosis.
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  • Masayuki SOMA, Yoshihisa MAEDA, Masahiro MURAKAMI, Shinpei KASAKURA, H ...
    1988 Volume 29 Issue 11 Pages 2024-2028
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    Tissue plasminogen activator (t-PA) antigen and plasminogen activator activity (PA activity) were investigated in 67 cancer patients with a variety of tumors, and compaired with age-matching controls.
    Our results show that t-PA antigen was signifficantly decreased in gastric, liver, pancreatic and rectum carcinoma withot metastasis, whereas it was significantly increased in the presence of metastasis. PA activity had no change in all cases.
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  • Takashi MACHII, Teruo KITANI, Kiyoji KIMURA, Isao MAEKAWA, Michiyuki M ...
    1988 Volume 29 Issue 11 Pages 2029-2036
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    Interferon-α (IFN-α) is highly effective in the treatment of hairy cell leukemia (HCL) in western countries. The efficacy of IFN-α in Japanese patients with HCL has not been determined, who have considerably different hematologic features from those of HCL patients in western countries. In the present study, the clinical effects of human lymphoblastoid interferon in 17 patients with HCL and 5 patients with lymphoproliferative disorders simulating HCL (tentatively designated as hairy cell leukemia variant) in Japan were tested.
    Fifteen of 17 HCL patients completed at least eight weeks of treatment and were evaluated: IFN-α was discontinued in two patients because of severe leukopenia and skin eruption, respectively. There were one complete and five incomplete remission, and overall response rate was 40%, which was significantly lower than those reported in western countries. The clinical, hematologic findings of 15 patients were compared to those of HCL patients in western countries. Only one patients, who achieved complete remission, had hematologic and immunophenotypic features entirely compatible with those of typical HCL in western countries. In contrast, the remaining 14 patients differed considerably from the formers and were classified in Japanese type HCL, a type of HCL found in most HCL patients in Japan. Two additional HCL patients showed more than 50% reduction in bone marrow hairy cell and some improvement of cytopenias, while none of five patients with hairy cell leukemia variant responded to IFN-α.
    These findings suggest that the difference in the efficacy of IFN-α between HCL in Japan and in western countries may be due to the difference in the type of HCL, although the relatively low sensitivity of Japanese type HCL to IFN-α remains unexplained.
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  • Junki TAKAMATSU, Motohiro HAMAGUCHI, Kazuo KAGAMI, Kazuhisa SUEHIRO, M ...
    1988 Volume 29 Issue 11 Pages 2037-2041
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    Four episodes of ovarian hemorrhage in the patients with 3 kinds of congenital bleeding tendency are reported. A diagnosis of congenital afibrinogenemia has been made prior to onset of ovarian hemorrhage and patient was treated with blood transfusion and fibrinogen preparations. Operations were successfully completed in the two patients with von Willebrand's disease and one congenital dysfibrinogenemia, whose diagnoses were made after surgery.
    Although recent advance in the diagnosis and treatment of congenital bleeding tendency, precise diagnosis have not been made in many patients and surgical procedures are still dangerous. So, one should consider the possibility of such a hemorrhage in such patients even though it is rare.
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  • Masanori SAITO, Hidesaku ASAKURA, Keiko ITO, Hiroshi JOUKAJI, Chika UO ...
    1988 Volume 29 Issue 11 Pages 2042-2047
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    Crosslinked fibrin degradation products (XDP) have been considered to be specific markers for differential diagnosis between DIC and fibrinogenolysis. In the present study, we quantitatively assayed the levels of XDP in normal subjects and patients with DIC and several other diseases by a newly developed assay procedure (particle counting immunoassay) based on the agglutination of latex particles coated with monoclonal antibodies. We then determined the usefulness of this assay procedure. The within-run and between-day reproducibilities of this method were satisfactory and the levels of XDP in plasma samples were closely correlated with those in serum samples obtained from the same patients. Comparing this method with previously established method, a good correlation was noted between these methods. The concentrations of XDP in many of normal subjects were less than 0.3 μg/ml, but moderately elevated XDP values occurred in some of 80 year old healthy subjects. In cases of DIC including patients with acute promyelocytic leukemia, the levels of XDP were markedly elevated. Mederate or slight elevation of XDP was seen in some cases of chronic cerebrovascular disorders, malignancy or diabetes mellitus. It was suggested that this assay procedure has a good reproducibility, is very simple and taken little times to diagnose DIC.
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  • Katsumi DEGUCHI, Eiichi IWASAKI, Mituya NOGUCHI, Ituko OHKUBO, Akira D ...
    1988 Volume 29 Issue 11 Pages 2048-2053
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    Thrombin-antithrombin III complex (TAT) in healthy subjects and patients with various diseases was assayed with solid phase enzyme immunoassay (ELISA) kit from Behringwerke A.G. (West Germany).
    Sixty healthy subjects had not taken drugs for over 2 weeks before the test, and ranged in age from 22 to 87 years (mean±standard deviation; 50.5±19.4). Their geometric mean plasma TAT was 2.02 μg/l (range from 0.90 to 4.5 μg/l).
    Concentrations over 4.5 μg/l were found in 10 of 19 patients with hematopoietic malignancy and solid tumor, and all 11 patients with DIC. In 8 of 9 patients with DIC, plasma TAT concentration at 24 hours after treatment with low molecular weight heparin was lower than that at the onset. In 7 patients receiving maintenance hemodialysis, plasma concentrations of TAT at the end of the dialysis session were higher than those immediately before dialysis, which platelets, coagulation and fibrinolysis factors are known to be activated by passage through the hemodialyzer.
    We conclude that TAT level is a sensitive indicator of latent activation of the clotting pathway.
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  • Susumu KAWAI, Yoshifumi YAMAMOTO, Masanori YANAI, Kunihiro NISHI, Keni ...
    1988 Volume 29 Issue 11 Pages 2054-2061
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    In order to investigate the clinical significance of the cell surface marker in acute non-lymphoblastic leukemia (ANLL), the leukemic blasts of 43 patients with ANLL were treated by 10 monoclonal antibodies and analysed these results in relation with response to therapy.
    These ANLL patients were registered to protocol ANLL 827 and 861 of the Children's Cancer and Leukemia Study Group.
    The percentage of leukemic cells that reacted to each antibody varied widly among patients.
    Expression of Mol was more commonly observed in AMMoL/AMoL (M4, M5) (80%) than in AML (M1, M2) (25%). MY7 and MY9 reacted to over 50% of both AML and AMMoL/AMoL, while the reactivity of Mo2 and MY4 was low (under 50%) in both groups.
    Expression of granulocyte-monocyte associated antigens didn't correlate to the FAB classification except for the higher frequency of expression of Mo1 antigen in AMMoL/AMoL than in AML.
    There was correlation between the expression of antigens and the prognosis of ANLL. Three myeloid antigens (Mo1, Mo7, MY9) predicted a low complete remission (CR) rate by induction chemotherapy; Mo1+ cases had a CR rate of 63%, while Mo1- cases had a CR rate of 82%. MY7+ and MY9+ cases had a CR rate of 63% and 58%, respectively, while MY7- and MY9- cases had a CR rate of 87% and 92%. The expression of MY7 or MY9 antigen was associated with a decreased continuous complete remission rate (34% and 30% at 24 months for MY7+ and MY9+ cases, respectively, vs 66% and 75% for MY7- and MY9- cases)
    These results confirmed the earlier reports on antigenic heterogeneity in ANLL. And it was considered that a further investigation is necessary to know whether the surface marker can predict the prognosis of ANLL independent of FAB classification.
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  • Yuji FUJII, Teruaki HONGO, Yoshio IGARASHI
    1988 Volume 29 Issue 11 Pages 2062-2067
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    Serial spectral EEG analysis in eight children with acute leukemia and non-Hodgkin's lymphoma was performed to investigate the influence of intermediate-dose methotrexate (ID-MTX) on the central nervous system (CNS). The EEGs, recorded before and 24h or 48h, one week and a few months after administration of ID-MTX, were analyzed as to the power average spectrum of the occipital basic activity using an ATAC-450 (NIHON KOHDEN). Although there was no patient whose serum and cerebrospinal fluid MTX-concentration exceeded the upper therapeutic limit, a transient but statistically significant slowing, such as about 0.7 Hz drop in the dominant frequency, was observed at 48 hours after the initiation of ID-MTX. No neurological symptoms were noted, and these data suggest that EEG alterations might be a reflection of subclinical CNS impairment. Therefore, serial EEGs might be a good early indicator for the detection of CNS impairment in children treated with ID-MTX.
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  • Masanori SAITO, Hidesaku ASAKURA, Chika UOTANI, Hiroshi JOKAJI, Ichiro ...
    1988 Volume 29 Issue 11 Pages 2068-2072
    Published: 1988
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    Leukocytes are though to participate in the progress of disseminated intravascular coagulation (DIC) caused by severe infection such as sepsis. We have had the impression that DIC rarely occurs at the nadir of leukocyte counts induced by intensive chemotherapy despite the complicating severe infection. In this study, we compared hemostatic parameters at the nadir with those during the recovery period to investigate the role of leukocytes in the coagulation system. The levels of serum FDP were higher in the cases who died during the recovery period than in the cases who died at the nadir, although they were within normal limits at the nadir in the former group. There was no difference in the severity of inflammation between the two groups. It was suggested that the hypercoagulality trends to occur during the recovery period, if there is still severe infection, rather than at the nadir.
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  • Tomoko SAITO, Shuichi MIYAWAKI, Kazuo NEMOTO, Hideaki SUGA, Kunihiko Y ...
    1988 Volume 29 Issue 11 Pages 2073-2078
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    A 69-year-old female was admitted to our hospital on February 6, 1984, because of fatigability, lymphadenopathy, progressive anemia and rash lasting for several months. Bone marrow examination, which had been performed a month before the admission, revealed a compatible finding with pure red cell aplasia. Since then, leukopenia and thrombocytopenia with bleeding tendency had developed. On admission, physical examination revealed hepatosplenomegaly, in addition to lymphadenopathy and purpura all over the body. Laboratory findings were as follows: Hb 5.2 g/dl, RBC 208×104l, reticulocytes 0‰, platelets 1.8×104l, WBC 3,600/μl and increased in polyclonal gammaglobulin. A biopsy specimen showed hypoplastic bone marrow with increased fibrous tissue. As immunological abnormalities, direct and indirect Coombs test, anti-nuclear antibody, anti-DNA antibody, anti-smooth muscle antibody and thyroid-microsome test were all positive. A diagnosis of Angioimmunoblastic lymphadenopathy was made on the basis of lymphnode biopsy findings and clinical features described above. She achieved clinical remission by administration of prednisolone and results of laboratory test including bone marrow findings were improved to near normal range. But, she was died of secondary bilialy liver cirrhosis and bronchopneumonia 10 months after in the course of tapering off the doses of prednisolone. At autopsy, there was universal depletion of lymphoid tissue with cellular reduction and thickning walls of the vessels in the lymphnodes.
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  • Noriko HASEGAWA, Hiroki HOSHINA, Hisao KUDO, Yasuhiro ENOMOTO, Yasuhid ...
    1988 Volume 29 Issue 11 Pages 2079-2085
    Published: 1988
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    We report in this paper a case of acute magakaryoblastic leukemia (AMKL) in Down syndrome accompanied by 3q-, showing tumor formation and myelofibrosis. The patient was an one and 9/12 year-old girl with Down syndrome. She was admitted to our hospital because of anemia and bleeding tendency.
    On admission, emaciation, hepatosplenomegaly and generalized lymphadenopathy were dominant. Peripheral blood was pancytopenic and white blood cell count was 3,400/μl with 14% blasts. The blasts with fine granules on light microscopic study were negative for peroxidase, positive for ACP, and PAS stainings. On electron microscopic study, the blasts had α-granules and small amount of demarcation membranes, and were positive for platelet peroxidase staining. By bone marrow biopsy, myelofibrosis and an excessive proliferation of atypical megakaryocytes were noted. Chromosomal analysis of peripheral blood disclosed 47, XX, del (3)(q21) and 47, XX, +21, der (8) t(8;1)(q24:q12).
    A diagnosis of AMKL was made, and prednisolone, vincristine, daunorubicin, cytosine arabinoside were administered, but they were not effective. A solid tumor appeared first at the right forehead at the age of two years, and then progressed to the upper and lower mandibulas, orbita and right thigh with an increase in size. She died of respiratory failure due to tumor obstruction when she was two and 3/12 years old. On post-mortem examination it was revealed that diffuse and nodular infiltrations of blast cells were detected in most of the visceral organs and bones, and formed tumors.
    Recent investigation revealed that the incidence of abnormality of each NO. 21 and No. 3 chromosome is high in AMKL, but the case having chromosomal abnormalities of both No. 21 and No. 3 as presented here is not reported previously. It is sugested that the abnormalities of the two chromosomes are reflected by this paticular clinical course.
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  • Hiroshi FUJII, Taira MAEKAWA, Kazuhiro NISHIDA, Youji URATA
    1988 Volume 29 Issue 11 Pages 2086-2090
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    A 67-year-old female was admitted because of bone pain in July, 1984. Based on the presence of anemia, the elevation of serum creatinine and calcium levels, the depression of serum immunoglobulins, Bence Jonces proteinuria (lambda type, 3.0 g/day), multiple osteolytic lesions and plasmacytosis in the bone marrow, a diagnosis of multiple myeloma of Bence Jones lambda type was made. After chemotherapy with cyclophosphamide and melphalan, her clinical status had been improved in August, 1984. In April, 1986, plasma cells in the bone marrow increased to 80%. The majority of plasma cells in her bone marrow had the indented or lobulated nuclei and some of them were also observed in the peripheral blood. Electron-microscopically, immature plasma cells with dilated cysternae of rough endoplasmic reticulums, multilobulated nuclei and numerous perinuclear cytoplasmic fibrils were observed. The majority of nuclear DNA contents of plasma cells, measured by cytofluorometry, showed the diploid DNA content and no differences existed between the nuclear DNA contents of plasma cells with lobulated nucleus and those with round nucleus. The chromosome numbers ranged from 52 to 53 and a 14 q+chromosome abnormality was observed in 18 of 19 karyotypes examined.
    The case presented here has suggested that the clonal selection caused by chemotherapy and/or perinuclear cytoplasmic fibrils may play a role in the development of nuclear lobulation.
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  • Touru OHTSUKA, Naoto CHIKADA, Satoru FUJII, Osamu SHIKATA, Norihiro AO ...
    1988 Volume 29 Issue 11 Pages 2091-2095
    Published: 1988
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    In general, plasmacytomas are tumors which produce monoclonal globulin. Multiple myeloma, in which punched out of bones, urinary Bence Jones protein, serum M-protein, etc., are recognized, is well known as a kind of plasmacytoma. However, single plasmacytomas of the bone and primary soft tissue plasmacytomas have been reported to occur in rare cases. Extramedullary plasmacytomas include secretory and non-secretory types. Primary plasmacytomas of the upper respiratory tract are frequently of the non-secretory type, and dignosis can often be achieved only by a histopathological examination. Recently, the authors encountered a case of extramedullary plasmacytoma developed in the right nasal cavity, and this was identified as IgA κ type by the enzyme-labeled antibody technique. The details of this case are reported below. together with a review of the literature.
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  • Kenji SUGA, Yoshinori SHIMAMOTO, Kazutoshi ONO, Masayuki SANO, Miwako ...
    1988 Volume 29 Issue 11 Pages 2096-2100
    Published: 1988
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    A 37-year-old female was admitted to our hospital in December 1983 for evaluation of severe anemia. At the time of admission, peripheral blood examination showed RBC 118×104l and Hb 3.7 g/dl. Reticulocyte count was 0.1%. The bone marrow aspiration showed normocellularity with abnormal lymphoid cells and a depletion of erythroid precursors (1.6% of bone marrow cells). A diagnosis of pure red cell aplasia (PRCA) was made. Anemia was recovered by corticosteroid therapy. She was discharged in March 1984. However, she was admitted again in December 1986 because of anemia and lymph node swelling in cervical and mediastinal region. From the finding of the biopsied cervical lymph node, non-Hodgkin lymphoma (diffuse, mixed) was diagnosed. After combination chemotherapy with vincristine, cyclophosphamide, prednisolone and adriamycin, the lymph node swelling decreased. After 4 months from the second admission, peripheral leukocyte count increased suddenly with abnormal lymphocytes. Abnormal lymphocytes in peripheral blood and lymph node swelling disappeared by chemotherapy. But pancytopenia developed subsequently. The case reports of PRCA associated with malignant lymphoma from the literature, including this case, were reveiwed and discussed.
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  • Tomoko MATSUOKA, Shizuo YOSHIMOTO, Hiroshi SONOBE, Yasushi KIYOKU, Mot ...
    1988 Volume 29 Issue 11 Pages 2101-2104
    Published: 1988
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    Two cases of non-African Burkitt's lymphoma in long-term remission are reported. Case 1. A 52-year-old man developed incisional hernia after appendectomy and underwent re-operation in April, 1984, when he was noted to have an ileocecal tumor, 4 cm in diameter. Then, ileocecal resection was performed and he was histologically diagnosed as having Burkitt's lymphoma. He was given postoperative combination chemotherapy consisting of cyclophosphamide, adriamycin, vincristine and prednisolone (CHOP) and has been in complete remission for more than 40 months. Case 2. A 27-year-old woman was admitted to Nagasaki University Hospital with a rapidly growing tumor in the right breast at 24 weeks of pregnancy in October, 1982. Radical mastectomy and Cesarean section were performed. Histological examination of the tumor revealed that she had Burkitt's lymphoma. Cell marker study showed that the tumor cells were positive for IgM, κ type immunoglobulin. Then, combination chemotherapy of CHOP was stated. She has been in complete remission for more than 60 months after surgery. Extensive resection of tumor and intensive postoperative chemotherapy appear to be the treatment of choice in selective cases of Burkitt's lymphoma.
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  • Shigehisa TAMAKI, Michiaki OHIWA, Yasuhiro UEMURA, Naoyuki KATAYAMA, M ...
    1988 Volume 29 Issue 11 Pages 2105-2110
    Published: 1988
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    A 16-year-old male was hospitalized because of submandibular lymphonode swelling and general fatigue. He was diagnosed as acute lymphocytic leukemia (ALL) which was morphologically characterized by L2 (FAB classification) and immunologically determined by T-cell type. A combination chemotherapy was carried out and successfully induced complete remission. 11 months later, he died from a intracranial bleeding with hematological relapse.
    In cytogenetic examination of pathological cells and normal lymphocytes in complete remission, constitutional chromosome anomalies of 47, XYY was observed in all of 20 metaphases examined respectively.
    His mother died of acute promyelocytic leukemia (APL-M3) 3-years before his admission in our hospital.
    These cases are suspected as familial leukemia, and this is the first case report of lymphocytic leukemia in XYY male with familial occurrence of leukemia.
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  • Yuri SUGIURA, Haruki NAKAMURA, Masako MINAMIHISAMATU
    1988 Volume 29 Issue 11 Pages 2111-2115
    Published: 1988
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    A 40 year old male was admitted to our hospital in August 1986, because of high fever and gingival swelling following the extraction of his teeth. On physical examination, anemia, purpura throughout his body and gingival ulcer were noted. He showed small testes and undeveloped penis. Hematological examination revealed acute myelocytic leukemia (M1) Chromosome study in peripheral blood cells showed 47, XXY, (7/20 cell) and 47, XXY, t(1;14)(p13;q32) (13/20 cell), whereas 47, XXY, (18/18 cell) in bone marrow cells. In spite of cytostatic treatment (BHAC-DMP, BHAC-AMP), complete remission was not achieved. He died after CNS leukemia finally of lung bleeding.
    Klinefelter syndrome is a disorder characterized by a 47, XXY karyotype that occurs in approximately 2/1,000 males. Until now thirteen cases with the combination of Klinefelter syndrome and acute leukemia are recorded in the literature. We here present these cases and discuss possible connection between the constitutional chromosome abnormality and acute leukemia.
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  • Kensuke USUKI, Yutaka KAWAMURA, Kinuko MITANI, Fuyuki ISHIKAWA, Akio U ...
    1988 Volume 29 Issue 11 Pages 2116-2120
    Published: 1988
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    A 19-year-old male with severe aplastic anemia suffered from acute appendicitis. Laboratory studies showed a leukocyte count of 200/μl and a platelet count of 2,000/μl, and he underwent appendectomy with massive platelet transfusion and high-dose administration of antibiotics. These provided complete resolution of acute appendicitis. In the past, conventional managements of acute abdomen in patients with pancytopenia were mainly conservative and nonoperative. However, advances in supportive technics such as transfusions of platelets and administrations of antibiotics have made surgical approach possible. This case suggests that a more aggressive surgical approach to the patients with severe aplastic anemia seems indicated.
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  • Junji TOMIYAMA, Yuji SATO, Toshitaka KOBAYASHI, Akira SHIBUYA, Haruhik ...
    1988 Volume 29 Issue 11 Pages 2121-2126
    Published: 1988
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    The case of a 45-year-old male with ALL (L2) who began to have acute mixed leukemia (AMxL) during maintenance chemotherapy, is reported. He had been on the maintenance therapy until March 1987, when he was readmitted to our hospital. On admission the hematological findings were as follows; hemoglobin 11.7 g/dl, platelet count 280×103l and leukocyte count 2.6×103μl with 4% blasts. The result of bone marrow (BM) aspiration showed that 90% were blasts (20.5% large blasts and 69.5% small blasts) negative for both myeloperoxidase and Sudan black B. Examination of the surface phenotype revealed 61.9% CD19+, 36.8% CD10+ and 21.7% CD33+ cells. After remission reinduction therapy 40% blasts were still present in the bone marrow (22% large blasts and 69.5% small blasts). The blasts were positive for both myeloperoxidase (17%) and Sudan black B (46.5%). Two parameter analysis with FACS revealed that CD33+19- cells were 45.8%; CD33-19+, 15.1%, and CD33+19+, 2.4%. Those findings indicate that the blasts were composed of two distinct populations of myeloid or lymphoid blast cells. Although gene analysis was not performed, this case was considered to be biclonal rather than biphenotypic AMxL. Then additional chemotherapy was given. However, the small blasts remained at 43.6% in the bone marrow and he died of septicemia in May 1987. Three cases of AMxL transformed from acute leukemia after chemotherapy had been reported in literatures. All of those including our case were resistant to chemotherapy.
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  • Yoshihito MORIOKA, Shinsaku IMASHUKU, Akane MIZUTANI, Koichi SAKATA, T ...
    1988 Volume 29 Issue 11 Pages 2127-2131
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    A 6-year-old boy with acute lymphocytic leukemia (common ALL, FAB L1), diagnosed in December 1984, developed interstitial nephritis during maintenance chemotherapy. He attained a complete remission, and after CNS prophylaxis (2,400 rads cranial irradiation with intrathecal methotrexate) he was receiving maintenance chemotherapy. In September 1986, he complained of thirst and polyuria. Urine volume was 1.5-6 1/day and its specific gravity was below 1.010. Vasopressin test and water deprivation test indicated nephrogenic diabetes insipidus. Hydrochlorothiazide was administered and urine volume was well controlled. In January 1987, the patient was given antibiotics because of fever due to chemotherapy-induced leukopenia and developed deteriorated renal function. The renal biopsy revealed edema and mononuclear cell infiltration in interstitial tissues and degeneration of tubular epithelial cells with intact glomeruli, confirming the diagnosis of interstitial nephritis. We speculate that direct renal tubular damage by anti-cancer agents like methotrexate may have caused interstitial nephritis in this patient.
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  • Yasuo KUWAZURU, Shuichi HANADA, Toshiaki UEMATSU, Takashi SAITOU, Tora ...
    1988 Volume 29 Issue 11 Pages 2132-2135
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    We present hereinafter a patient, initially treated for Pneumocystis carinii (P. carinii) pneumonia and terminated adult T-cell leukemia (ATL).
    A 56-year-old male was admitted for dry cough and exertional dyspnea in March, 1984. His X-ray examination of chest and transbronchial lung biopsy revealed P. carinii pneumonia. Sulfamethoxazol-trimetoprim showed good response and his all symptoms disappeared. Immunological examinations were as follows: Anti ATLA was positive. PPD reaction was negative. Surface phenotype of blood lymphocytes showed decrease of OKT4 positive cells and inversion of OKT4/OKT8. A few abnormal lymphocytes with nuclear indentation or lobulation were seen in the peripheral blood.
    After about 1 year, he was again admitted in our hospital for marked lymphocytosis with nuclear abnormality, hepatomegaly and lymphadenopathy. The diagnosis of ATL was made. Combined chemotherapy with supportive therapy was poorly effective and he died with intestinal bleeding.
    This case is very noteworthy from the point of view of immunological function of human T-cell leukemia virus carrier before outbreak of ATL.
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  • Minoko TAKANASHI, Michihiko MASUDA, Toshiko MOTOJI, Kazuo OSHIMI, Hide ...
    1988 Volume 29 Issue 11 Pages 2136-2140
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    A case of acute graft-versus-host disease (GVHD) following chemotherapy and platelet transfusions is presented.
    This 43-year-old female with relapse of acute myeloblastic leukemia was successfully treated. After consolidation chemotherapy of high-dose cytosine arabinoside and daunorubicin, unirradiated platelets were transfused three times. Platelets were obtained from her sister, because the patient had become refractory to random donors' platelet transfusion, and direct cross match test was negative between patient's serum and sister's lymphocytes. Twenty-second days after chemotherapy or 7 days after the first platelet transfusion, high fever and diarrhea developed, and were followed by erythroderma and liver dysfunction. Drug allergy was suspected, and all the antibiotics were discontinued. After transient recovery of granulocytes, however, pancytopenia deteriorated. Biopsy of the bone marrow and skin was performed, and their histologic findings were consistent with those of transfusion-associated GVHD. High-dose methylprednisolone, cyclosporin A and antilymphocyte globulin were given, but ineffective. She died of severe pancytopenia and intestinal bleeding. HLA typing of the family members revealed that the patient had A11, A33, B15, B44, Cw4, and her sister A33, B44, DRw13. A33 and B44 homozygous in her sister were histocompatible with one of the haplotype of the patient's. This may be the most likely factor for the occurence of GVHD.
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  • Naomi SAKIYAMA, Reiko UESHIMA, Yoshio OURA, Osamu KUNITOMI, Hiroko TAN ...
    1988 Volume 29 Issue 11 Pages 2141-2144
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    A 25-year-old female, who had often suffered from excessive menstruation since 11 years old, was admitted to our hospital for the control of hemostatic state at her delivery. She was born of a consanguineous marriage and diagnosed to have congenital factor VII deficiency at 24 years old. On admission, she was normal in physical examination and the prothrombin time was prolonged due to the decrease of factor VII activity reaching to 3.2%. Application of heat-treated factor VII concentrates (Immuno, Austria) was tried for hemostasis at her delivery. Two thousand units of these factor VII concentrates were given to the patient about 1 hour before her delivery. Her factor VII activity level elevated rapidly to 107.6% during the first 30 min, then decreased gradually and maintained above 20% for 6 hours. During this time, she delivered a male baby without excessive bleeding. His factor VII activity was 17.5%. Any complications were not found in the use of these factor VII concentrates.
    The supplemental administration of the heat-treated factor VII concentrates was thought to be safely effective for hemostasis at delivery, especially in such a factor VII deficient patient suffering from a severe hemorrhage tendency.
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  • Norimitsu KADOWAKI, Hiroshi KAMESAKI, Yohichiroh OHNO, Hiroyuki AMANO, ...
    1988 Volume 29 Issue 11 Pages 2145-2151
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    A 70-year-old man who had been observed under the diagnosis of refractory anemia with excess of blasts (RAEB) was admitted to our hospital because of fever and wheezing. Physical examination revealed mild hepatomegaly without splenomegaly. Laboratory data showed moderate anemia and thrombocytopenia. Bone marrow was hypocellular with dysplasia of all three hematopoietic cell lines. Five months after the diagnosis of RAEB, the peripheral monocyte count increased more than 1,000/μl, indicating the transformation of RAEB into chronic myelomonocytic leukemia (CMML). At the end stage, the peripheral monocyte count exceeded 100,000/μl. The urinary excretion of lysozyme and serum lactic dehydrogenase were markedly elevated. Most monocytes were in mature form but were poorly stained with α-naphtyl butyrate esterase. Karyotypic analysis which showed a normal pattern on admission revealed complex abnormalities after the evolution of RAEB into CMML. These findings indicate that the monocytes had the neoplastic nature. The anabolic steroid, vitamin D3, and low dose cytarabine had no benefit. He died from respiratory failure seven months after the diagnosis of RAEB. Autopsy revealed extensive infiltration of leukemic cells. This case is informative in regard to the transformation of RAEB into CMML, with subacute course, in association to the clonal evolution.
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  • Kuniaki ITOH, Kiyoshi HIRUMA, Hisashi WAKITA, Nobuyuki ENDOH, Tadahiko ...
    1988 Volume 29 Issue 11 Pages 2152-2157
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    A 34 year-old male was admitted on Dec, 27, 1985, due to an apparent fever. The white blood cell count was 15,400/μl with a differential count of 61% blasts. The red blood cell count was 251×104l. The Hb was 8.3g/dl. The platelet count was 2.4×104l. Bone marrow aspiration revealed hypercellularity with 85.2% blasts. The blasts were positive for peroxidase, naphthol ASD chloroacetate, and alpha naphthyl butyrate esterase. Acute myelomonocytic leukemia (M4) was diagnosed. The patient entered into complete remission under BHAC-DMP therapy. The patient's father, aged 65, who had been operated upon for gastric cancer 5 years ago, matched the patient's HLA. The fathers' hematological data and marrow progenitor cells were within normal limits except moderate decreased CFU-GM. Although the patient was in a relapsed state from May, 1986, bone marrow transplantation was performed on June, 9, 1986. After high dose cyclophosphamide administration (60mg/kg×2days) and TBI (3GY×4days), the patient received 3.4×108/kg nucleated marrow cells. The white blood cell count recoverd to 1,000/μl on day 35. Marrow engraftment was detected by a change of blood type (O→A). The patient remained in complete remission for 23 months after the BMT.
    Our findings suggested that the recovery of the granulocyte count may in fact be slower in elderly donors.
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  • Keisuke TOMII, Hiroshi KAMESAKI, Yohichiroh OHNO, Hiroyuki AMANO, Taka ...
    1988 Volume 29 Issue 11 Pages 2158-2162
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    A 39-year-old man was admitted to Tenri Hospital, because of multiple cutaneous nodules, which were located in his face, trunk and extremities. His Hb was 16.0g/dl; Plt, 289×103l; WBC, 8,200/μl with normal differential counts. Blood chemistry was normal except for high serum lysozyme. There was slight increase of immature monocytes (22%) in the sternal marrow, but not in the iliac marrow. The cutaneous nodules were within 3 cm in diameter, and were composed of diffuse atypical cells in the dermis and subcutaneous tissues. These cells were diffusely stained with NaF sensitive α-naphthyl butyrate esterase and acid phosphatase, were positive for My4 and My7 antigens, but were negative for T cell-, B cell-, and interdigitating reticulum cell markers. Electron microscopy disclosed nucleus with prominent nucleoli, myeloperoxidase positive granules (<0.2μm) and well developed smooth-ER. Therefore, atypical cells of the skin were of immature ones of monocyte origin, indicating that the patient should be diagnosed as monocytic aleukemic leukemia cutis (monocytic sarcomaof the skin). Effect of anti-leukemic chemotherapy was poor, but total skin electron beam irradiation showed dramatic effect on skin tumors. However, the patient proceeded to overt acute monocytic leukemia 12 months after admission and skin tumors relapsed. He died of sepsis 5 months thereafter.
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  • Yuji SATO, Yasuhiro YODA, Toshitaka KOBAYASHI, Junji TOMIYAMA, Tsukasa ...
    1988 Volume 29 Issue 11 Pages 2163-2167
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    The case of a 48-year-old female with acute myelocytic leukemia (AML) M2 associated with an abnormal karyotype, 46 XX, t (9; 13) (q34; q12) is reported. She visited our hospital on June 20, 1985 for anemia. Physical examination was unremarkable except for some petechiae on the extremities. CBC showed severe anemia, thrombocytopenia and leukopenia with 1% myeloblasts. In the bone marrow (BM), myeloblasts were 24.6% including those with Auer bodies. Significant morphological abnormalities were noted in the myeloid, erythroid and megakaryocyte series. A week later, the repeated BM study showed myeloblasts to be 30.2%. Then the diagnosis of AML M2 advanced from myelodysplastic syndrome (RAEB-T) was made. The initial chromosome analysis before the chemotherapy revealed the abnormal karyotype in all cells examined. After the treatment with low dose ara-C, the cells with the abnormal karyotype decreased and eventually disappeared when a complete remission was obtained. The chromosome analysis using the G-banding technique revealed the karyotype to be del (6) (p23), t (9; 13) (q34; q12). This type of abnormality has never been described previously. The treatment with low-dose ara-C successfully decreased the abnormal clone. This indicates that low-dose ara-C was cytocidal for the abnormal cells.
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