Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
Volume 27, Issue 10
Displaying 1-34 of 34 articles from this issue
  • —Evaluation of Whole Blood Platelet Aggregation—
    Hidekazu KAWAMURA, Hiroyuki KODAMA, Genji TAKEUCHI, Keiju KOHSAKA, Sad ...
    1986 Volume 27 Issue 10 Pages 1815-1822
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    The effect of dilazep dihydrochloride (dilazep) on platelet functions was evaluated in ten elderly patients with ischemic heart disease.
    The patients were given oral doses of 300 mg of dilazep daily, and in each case, both before treatment and two and four weeks after the start of treatment, a blood sample was withdrawn two hours after administration of the drug.
    Administration of dilazep resulted in an inhibition of platelet adhesiveness (P<0.05), a decrease in plasma thromboxane B2 level (P<0.01), and a tendency of decrease in plasma β-thromboglobulin and platelet factor 4.
    In whole blood platelet aggregation induced by ADP 5μM using impedance method, an inhibitory effect of the drug was observed immediately after venipuncture (P<0.05) but not observed one hour after venipuncture in tests induced by ADP 5 μM and collagen 2.5 μg/ml.
    Our results demonstrate that an antiplatelet effect of dilazep in vivo was beneficial, but it was observed only immediately after venipuncture in whole blood platelet aggregation tests in vitro.
    From this observations, it is necessary that a number of platelet function studies be undertaken to evaluate properly the antiplatelet effect of a drug. Moreover, whole blood platelet aggregation using impedance method examined immediately after venipuncture is very useful in evaluating such an effect of a drug.
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  • Kenji SHINOHARA, Katsunori YAMADA, Masamitsu INOUE, Youichi AZUNO, Mas ...
    1986 Volume 27 Issue 10 Pages 1823-1828
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    The bolus methylprednisolone therapy was ineffective for the treatment of our patients of aplastic anemia.
    However, the use of anabolic steroid or prednisolone after bolus methylprednisolone therapy, or the concomitant use of anabolic steroid and prednisolone initially from the time of diagnosis, markedly improved the pancytopenia; good and partial responses of 77% and prompt hematological recovery after 3 to 6 months.
    The clinical responses to these treatments were not always correlated with the results of the co-culture study in vitro for the immunological suppression of hematopoiesis.
    These results suggested that the responses may partly be due to the action of corticosteroid to the microenvironment of the bone marrow as the pre-conditioning of hematopoiesis.
    The side effect of these therapies was the impaired glucose tolerance in a small number of patients.
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  • Mayumi MORI, Yuji ZAIKE, Toshio TAKEDA
    1986 Volume 27 Issue 10 Pages 1829-1833
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    Periphetal lymphomono-nuclear cells in 48 institutionalized patients with Down's syndrome (DS), ranging from 8 to 48 years old, were investigated on lymphocyte subpopulations by using various monoclonal antibodies. The proportions of the lymphocytes reacting with anti-Leu4 and anti-Leu2a antibodies were significantly increased in DS, comparing with healthy controls. The percentage of the Leu7 positive cells was also increased in DS, without statistically significance. The positive correlation between Leu4 and Leu2a was demonstrated. It was suggested that the increase of the Lew4 positive cells was accounted for by the increase of the Leu2a positive cells. The increase of the Leu4 and Leu7 positive cells in DS revealed remarkably associated with the advancement of age.
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  • —Impaired Aggregation in Response to A23187 with Normal Response to Arachidonic Acid—
    Tateo SUGIYAMA, Minoru OKUMA, Fumitaka USHIKUBI, Kenji KANAJI, Shigeki ...
    1986 Volume 27 Issue 10 Pages 1834-1842
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    Functional, ultrastructural and biochemical studies on platelets were perfomed in a patient who had been reported to have a novel hereditary platelet function disorder. Aggregation responses to ADP, epinephrine, collagen, platelet activating factor, STA2 [a thromboxane (TX) A2 analogue], U-46619 [a prostaglandin (PG) endoperoxide analogue] and the Ca ionophore A23187 were deficient, but normal aggregation was induced by arachidonic acid (AA), PGH2, TXA2, thrombin, ristocetin and TPA (12-0-tetradecanoyl phorbol-13-acetate) which induces platelet functions by direct activation of protein kinase C. Platelet granules, serotonin uptake, contents of adenine nucleotides and glycoprotein profiles were all within normal limits, but ultrastructural studies demonstrated decreased numbers of mitochondria and increased accumulations of glycogen particles as well as of lipid droplets. Platelet AA metabolism in response to exogenous AA and thrombin was normal, but TXB2 production bycollagen-stimulated platelets was decreased, suggesting a defective collagen-induced liberation of AA from the membrane phospholipids. However, AA liberation was normal when the platelets were stimulated with thrombin. STA2, the Ca ionophore ionomycin and thrombin induced normal increase in the cytoplasmic Ca2+ concentration ([Ca2+]i) as estimated by quin 2. These findings suggest that the defect in the patient's platelet consists in utlization of cytoplasmic Ca2+ for secretion and aggregation rather than Ca2+ mobilization. Thus, the most likely defective sites could be the pathway which results in agonist-induced AA liberation after normal [Ca2+]i elevation rather than the abnormal phospholipase enzymes per se.
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  • Hiromasa YAMAUCHI, Hitoshi KURABAYASHI, Hitoshi KATAHIRA, Morio SAWAMU ...
    1986 Volume 27 Issue 10 Pages 1843-1849
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    Thiamphenicol (TAP) was administered orally to 10 patients with polycythemia vera for 14 days at a daily dose of 1 g in a total of 22 courses to evaluate its clinical usefulness in the management of this disease. Reticulocytes decreased markedly and hemoglobin became depressed by a mean of 1.4 g/dl on day 14. Likewise, hematocrit and RBC fell by 6.1% and 560×103/μl, respectively. Hemoglobin returned to pretreatment levels within 4 weeks after cessation of treatment. WBC also decreased by 2,900/μl, but neutrophil percentage did not change significantly throughout the observation period. Platelet showed a most remarkable reduction to 58% of pretreatment values on day 14 but full recovery was observed one week later. Bone marrow cellularity, megakaryocyte number and erythroblast percentage all decreased significantly by TAP. Mild stomatitis and gastrointestinal symptoms occurred in 4 courses.
    TAP may be listed as one of the useful therapeutic approaches in selected patients because of its rapid, reversible and well predictable myelosuppression.
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  • Setsuko EDAMURA, Souichi KOIKE, Masamichi SASAKI, Naomi TOMONO, Kaori ...
    1986 Volume 27 Issue 10 Pages 1850-1855
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    Chromosomal analysis on T cell malignancies including 10 cases of adult T cell leukemia and 6 cases of nonendemic T cell malignant lymphoma was performed comparing with 25 cases of non T cell malignancies.
    The structural rearrangements in T cell malignancies were most commonly found in No. 7 (63%) and NO. 12 (50%) in contrast with 28% and 16% respectively in non T cell malignancies. No consistent break point in No. 7 was found, but the translocation at band q24 in N. 12 was highly characteristic with a frequency of 63% (5/8).
    This abnormality in No. 12 could be related to the oncogene, k-ras-2, which is known to be located on the No. 12. Therefore 12q24 thought to be a clonal marker in T cell malignancies as 14q32 in non T cell malignancies.
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  • —Altered Central Nervous System Pharmacology of MTX with Meningial Relapse of Childhood Leukemia—
    Hatsufumi MATSUOKA, Kuniaki SASAKI, Takeo FUJIMOTO
    1986 Volume 27 Issue 10 Pages 1856-1861
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    The cerebrospinal fluid (CSF) pharmacokinetics of methotrexate (MTX) after high-dose intravenous infusion was investigated in 15 children with acute lymphoblastic leukemia (ALL).
    The concentrations of MTX in CSF and serum were monitored simultaneously at 6-hr and 24-hr after the initiation of MTX infusion for 47 times of infusion.
    The concentration of MTX in CSF was ranged from 0.5 μM to 4.1 μM with dosage between 1.4 and 5.6 g/m2.
    The transportation of MTX from systemic circulation to CSF was evaluated with the MTX CSF/serum % concentration ratio.
    The CSF/serum % concentration ratio during MTX infusion of patients with central nervous system (CNS) leukemia (1.45) was significantly higher (P<0.001) than that of patients without leukemic CNS involvement (0.63).
    Previous skull irradiation before 1.5∼2.0 years, had no influence on the MTX CSF/serum % concentration ratio.
    These data suggested leukemic infiltration in meninges accelerated the transport of MTX from circulation to CSF. The monitor of CSF/serum % concentration ratio during MTX infusion was considered to be essential not only for setting up the safety and appropriate schedule of high-dose MTX therapy, but also for predicting the CNS leukemia.
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  • Yasuhiko IKEDA, Yuichi SATO, Norio ONODERA, Eturo ITO, [in Japanese], ...
    1986 Volume 27 Issue 10 Pages 1862-1868
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    Primary thrombocythemia is rare in children, while many cases of the disease have been well noted in the middle age. Now we report two cases of the disease of children.
    In case 1, she is a ten-years old girl and she was first pointed out thrombocytosis when she was ill with strepto cocosis. Her peripheral platelet count was from one to two million per cubic millimeter and giant platelet or platelet aggregation was observed. In platelet function, ristocetin and epinephrine added platelet agglutination was decreased.
    In case 2, she is a nine-years old girl, and pointed out thrombocytosis when suffering from urinary tract infection. Peripheral platelet count was from one to two millions per cubic millimeter. Giant platelet was observed and ADP and epinephrine added platelet agglutination was decreased.
    Both cases showed no abnormal findings in electron micro scopic examination of platelets. On in vitro colony assays with bone marrow specimen, some colonies were formed in both cases without addition of colony stimulating factors in CFU-E and CFU-C. This data suggests the possibility of autonomous proliferation occurs at the level of pluripotent stem cell in this desease. At present time, we have treated them with dipyridamole for the control of hypercoagulavility.
    They are doing well for 39 months in case 1 and 42 months in case 2.
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  • Suzuaki SHIMIZU, Masataka OKAMOTO, Yoshitaka ONO, Masami HIRANO
    1986 Volume 27 Issue 10 Pages 1869-1873
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    An 18-year-old female with CML first developed a lymphoid crisis characterized by marked anemia, moderate thrombocytopenia and appearance of TdT-positive lymphoblasts in the bone marrow (97%) and the peripheral blood.
    Complete remission (CR) induced by VP therapy lasted for 16 months to be followed by the 2nd blast crisis, in which peroxidase-positive myeloblasts predominated. BHAC-AMP therapy induced marrow aplasia, which was then repopulated by lymphoblasts. The 2nd CR was obtained by VP and MTX-asparaginase sequential therapy. During this CR, pre-B cell CNS involvement occurred and was successfully treated. Two months later thrombocytosis occurred, gradually reaching the peak value over 5 million/cmm. Bone marrow aspiration revealed 40% myeloblasts, numerous megakaryocytes including micromegakaryocytes, and sheets of platelet clumps. Bone marrow soon became difficult to aspirate and needle biopsy showed foci of myelofibrosis. She died 39 months after the onset of the first blast crisis. Autopsy revealed infiltration of blasts in the liver, spleen, bone marrow and other organs and areas of necrosis in the bone marrow.
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  • Yoshitaka AOYAGI, Akira KOIKE, Yoshio TERADA, Masaaki KANAYAMA, Tatsu ...
    1986 Volume 27 Issue 10 Pages 1874-1879
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    A 77-year-old female presented in November 1984 with a one-month history of general malaise and low grade fever. On admission, the spleen was slightly enlarged or liver were not palpable. The peripheral blood showed marked leukocytosis with mild anemia and no tear-drop poikilocytosis. A few blast cells were myeloperoxidase-positive on electron microscopy. Repeated bone marrow aspirations were unsuccessful, and needle biopsy revealed a marked myelofibrosis accompanied with proliferation of three hematopoietic series. The diagnosis of “acute myelofibrosis” was made on the base of clinical and pathological criteria. During a few months after admission, she was treated with vincristin and prednisolone followed by OK-432 and low-dose cytosine arabinoside because of progressive pancytopenia. She died of sepsis caused by pneumonia in February 1985, shortly after hematological remission was achived. At autopsy, the bone marrow showed marked regression of fibrosis. Mild splenomegaly was found, but there was neither infiltration of leukemic cells nor extramedullary hematopoiesis in the liver and spleen.
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  • Tsuneo NINOMIYA, Yoshimi TSUDA, Masuhide MIYAO, Kenichi NISHIKAWA, Sho ...
    1986 Volume 27 Issue 10 Pages 1880-1885
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    Patient was the first of identical twin, 1,750 g products of an uncomplicated 36 weeks gestation to a healthy 28 years old mother. Her twin sister was the 1,950 g of birth weight. A single placenta was observed and monozygosity was diagnosed. She had been well developed until 17 months old at the time of initial diagnosis of acute lymphocytic leukemia in April 1977. Remission was induced with vincristin and prednisolone. She was received CNS prophylaxis with intrathecal injection of methotrexate. In December 1977, lumbar puncture showed mononuclear cells 7/3 μl and 10% lymphoblasts were identified by gravity sedimentation. This subclinical CNS leukemia was successfully treated with intrathecal injection of methotrexate and cytosine arabinoside which maintained every 12 weeks for 4 years, in addition to oral maintenance drugs. As repeated bone marrow examination and lumbar puncture showed no evidence of active disease, therapy was stopped in March 1983.
    In May 1984, 1 1/6 year after cessation of therapy, she developed gingival bleeding. Bone marrow examination showed total replacement with leukemic lymphoblasts. A second remission was induced with vincristin, L-asparaginase and prednisolone. She has been in complete continuous remission.
    To measure IQ she was tested using Wechsler Intelligence Scale for Children-Revised (WICSR) and scored significantly lower, being made by comparison with her twin sister and another leukemic patients. Though it is difficult to identify the factor that may be associated the lower level of her intellectual function, longer duration of intrathecal chemotherapy in addition to maintenance drugs was suspected. Serial tests of intellectual function have to be examined to develop the better treatment to childhood acute lymphocytic leukemia.
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  • Yoshitaka HISHITANI, Tadashi KANOH, Hitoshi MURAO, Koichi KAKEHI, Tosh ...
    1986 Volume 27 Issue 10 Pages 1886-1890
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    A case of multiple myeloma (IgG-κ) associated with phagocytic plasma cells and a variety of abnormal morphologic features of bone marrow cells is demonstrated.
    A 64 year old woman, in whom multiple myeloma had been diagnosed in 1977, was initially treated with cyclophosphamide and prednisolone. Three years later, cyclophosphamide therapy was stopped because of remission, and during next 4 years, she was in good clinical condition. in Oct. 1984, she was hospitalized because of back pain and an increase of M-component in serum. Bone marrow aspiration revealed 16% typical myeloma cells without phagocytosis and no abnormal morphologic features of bone marrow cells. The patient was given melphalan 6 mg/day intermittently (Total 60 mg). After this treatment, 2% of myeloma cells demonstrated phagocytosis of platelets. One third of myeloma cells nuclei showed morphological abnormalities such as Rieders form or hypersegmentation. Some of neutrophils and a number of erythroblasts showed fragmentation nuclei or hypersegmentation nuclei. Ringed sideroblasts were also seen.
    This case is considered as a myelodysplastic syndrome because of these abnormal morphologic features of bone marrow cells and ringed sideroblasts. These features may be associated with the chemotherapy such as melphalan or cyclophosphamide.
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  • —Report of Two Cases and Review of Literature—
    Tomomitsu HOTTA, Hisami YAMAO, Taizi KATO, Takashi MURATE, Makoto UTSU ...
    1986 Volume 27 Issue 10 Pages 1891-1898
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    Two cases of aplastic anemia transforming to myelodysplastic syndromes via hematological remission were reported. Case 1, a 47 year-old housewife was diagnosed severe aplastic anemia at 41 years of her age. Complete remission was attained following androgen therapy 3 years after diagnosis. Two years later anemia reappeared with monocytosis. Her bone marrow showed hyperplasia with prominent dyshematopoiesis. The anemia was refractory to androgen anymore. Case 2, a 28 year-old female previously diagnosed as moderate aplastic anemia and being in remission after androgen therapy for 4 years was admitted to our hospital because of anemia and bleeding tendency. Laboratory data showed high LDH level and ineffective erythropoiesis.
    We also reviewd 21 cases in the literature transforming from aplastic anemia to myelodysplasia through remission similar to our cases and proposed a concept of aplastic anemia-myelodysplasia syndrome.
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  • Hiroshi KONO, Yoshio OKUBO, Kazuhiko YOSHIMURA, Katsura OZAWA, Toshihi ...
    1986 Volume 27 Issue 10 Pages 1899-1903
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    An autopsy case of T cell chronic lymphocytic leukemia with gastric cancer is reported.
    A 74 year-old male was admitted to our clinic on February 19, 1979 because of leucocytosis in the peripheral blood which he had pointed out for ten years prior to admission.
    He had no complaint, and physical findings were essentialy normal except for slight bilateral cervical lymphadenopathy and hepatomegaly. Laboratory examinations revealed the marked leucocytosis (32,200/cmm) in the peripheral blood with predominant increase in matured small lymphocyte-like cell (79.5%), which were identified as T cell lymphocyte using immunological analysis.
    Histological findings of the biopsied specimen of the right cervical lymphnode were compatible with those of chronic lymphocytic leukemia.
    He was discharged on April 30, 1979 without specific treatment, and was observed as an outpatient there after.
    Three years later, the patent was readmitted with complaints of anorexia, nausea, abdominal pain and bloody stool on April 30, 1982.
    He was diagnosed complicating of gastric cancer (poorly differenciated adenocarcinoma) by upper gastrointestinal truct series examinations. The patient died on May 25, 1982, and the clinical diagnosis was confirmed by autopsy.
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  • Satoru TANAKA, Ryuichi AMAKAWA, Haruo NISHIMURA
    1986 Volume 27 Issue 10 Pages 1904-1908
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    A 25-year-old housewife was transferred to our hematologic department with severe genital hemorrhage and bleeding of gums, 5th day after delivery. Physical examination revealed anemia. The white blood cell count was 1,470/cmm and the platelet count was 29,000/cmm. Atypical promyelocytes were 63% of peripheral WBC and 82% of bone marrow cells. Normal ESR, hypofibrinogenemia and a high level of FDP were observed.
    The therapy was started under a diagnosis of acute promyelocytic leukemia, but she died, 75 days after delivery. Autopsy findings revealed partial proliferation of leukemic cells in the bone marrow. Marked bleeding in several organs and fibrin thrombi in the glomerular capillaries were noted.
    To date the child is healthy.
    Including our case, 20 cases of pregnancy with acute promyelocytic leukemia were reported in Japan since 1969. Two cases were not received chemotherapy during pregnancy. Hematologic examinations showed the findings of leukemia in none of infants.
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  • Tadahiko IGARASHI, Tadaaki MIYAMOTO, Kuniyuki OKA, Naoyoshi MORI
    1986 Volume 27 Issue 10 Pages 1909-1915
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    We described a rare case of follicular lymphoma producing IgG λ type M-protein and Bence Jones protein.
    A 64 year old male patient was admitted to the hospital of NIRS in Chiba because of leukocytosis and abdominal tumor. Diagnosis of follicular lymphoma medium sized cell type was made on his large tumorous mesenteric lymph node by laparotomy biopsy.
    On admission peripheral blood examination showed WBC 18,600/μl with 36.5% leukemic lymphoma cells. Bone marrow aspiration yielded normal cellularity containing 54.6% lymphoma cells. The PAP method observations of lymph node and peripheral leukemic cells showed that the lymphoma cells were positive with anti-IgG and anti-λ sera. Immunoelectron microscopic observation revealed that the positive reaction for IgG and λ was demonstrated in the nuclear envelope, rough endoplasmic reticulum and on the surface of these lymphoma cells, clearly indicating that these cells were producing IgG. In accordance with these findings the laboratory data showed IgG λ type M-proteinemia and Bence Jones proteinuria.
    As lymphoma cells were proved to invade to the liver and to the bone marrow, chemotherapy with adriamycin, cyclophosphamide, vincristine and prednisolone was started, but these drugs were ineffective. Next, melphalan was used alone successfully. Nearly complete remission with a little amount of residual leukemic cells in the bone marrow, was achieved after additional radiotherapy against tumor and swollen lymph nodes.
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  • Susumu NISHINARITA, Iwao SASAKI, Masaru HIRANUMA, Yoshihisa SUGAI, Tak ...
    1986 Volume 27 Issue 10 Pages 1916-1921
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    A 68-year-old male with a blastic crisis of chronic myelogeneous leukemia was admitted for the chemotherapy.
    He had a marked splenomegaly but neither edema nor dehydration. On hematologic examination anemia, thrombocytopenia and an appearance of a lot of immature cells and basophils in peripheral blood were noted. The other laboratory tests including serum electrolyte and renal function showed no abnormalities.
    The patient was given 40 mg/m2 of prednisolone for 4 days and 1.4 mg/m2 of vincristine (VCR) on the day 1 and the day 7. During the following week, the improvement on hematologic examination was observed, however, his appetite decreased markedly and severe fatigability appeared. At the day 14, low serum sodium and chloride, low plasma osmorality, high excretion of sodium and chloride in the urine, and high urine osmorality were observed. Serum sodium reached to the bottom of 100 mEq/L at the day 19.
    By the day 50 after admission, the patient recovered from hyponatremia with severe water restriction and the oral medication of demecrocycline. In the following hospital day, the patient was controlled with prednisolone and vindesine sulfate (VDS). The first injection (0.5 mg/m2) and the following three times injection (1.4 mg/m2) of VDS of every 3 or 4 weeks had no effect on the serum electrolyte and osmorality.
    The blastic crisis of CML seems to be well controlled with VDS and prednisolone as well as VCR and prednisolone. The patient has no complaint and is followed up as an outpatient.
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  • Yumiko TAKEUCHI, Yasunobu NONAKA, Yoshimi SAITO, Tsuneaki SUGIMOTO
    1986 Volume 27 Issue 10 Pages 1922-1927
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    Two cases of red cell aplasia with multiple immunological abnormalities that developed after resection of thymoma are reported.
    A 71 year-old woman was admitted because of anemia and neutropenia in August, 1985. In April, 1985, thymectomy was performed for the major tumor of a lymphocytic type thymoma, remaining pleural metastais unresected. On admission, RBC was 316×104/mm3, reticulocytes 0% and WBC 700/mm3. Bone marrow aspiration revealed complete loss of erythroid components and myeloid hypoplasia. Hypogammaglobulinemia was present and anti-nuclear factors, anti-microsome and antithyroglobulin antibodies were positive. 70% of lymphocytes were Leu 2a positive. After short term of spontaneous remission, anemia and neutropenia developed again. The patients died of pneumonia without knowing the effects of anti-lymphocyte serum.
    The other case was a 46 year-old man, who developed pure red cell aplasia 6 years after resection of a lymphoepithelial type thymoma. Thymoma relapsed 3 years after the diagnosis of PRCA, and it was surgically treated again. Anti-nuclear factors, anti-erythroblast antibodies and a false positive test for syphilis were detected. Anemia did not respond to medical treatment and the patient died of acute myocardial infarction.
    In both cases, autopsy failed to show any remained thymoma.
    Anemia developing after thymectomy is discussed with a review of literaure.
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  • Hideaki MIYAMOTO, Tetsuo NAGATANI, Junko MIYAKAWA, Hiroshi NAKAJIMA, S ...
    1986 Volume 27 Issue 10 Pages 1928-1933
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    We reported a case of smoldering adult T-cell leukemia. The patient had been suffered from Raynaud phenomenon, cutaneous sclerosis, lymphadenopathy and abdominal fullness in March 1983, and was thought as a kind of collagen diseases, for example, PSS. Eight months later, about 10% atypical cells (so called flower cells) appeared in her peripheral blood. Antibody to ATLA and proviral DNA of HTLV-I were positive at that time. From these data, she was diagnosed as ATL. In September 1984, a crisis occurred, and she died of pneumonia in January 1985, in spite of 4-course VEPA therapy. The autopsy was not done. And herpes zoster and controllable diabetes inspidus occurred in the course.
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  • Tetsuji KOYAMA, Yosio OURA, Tokuo NAKAJIMA, Eizo KAKISHITA, Kiyoyasu N ...
    1986 Volume 27 Issue 10 Pages 1934-1937
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    A 28-year-old female, who suffered from thrombotic thrombocytopenic purpura (TTP) on the 14th week of her first pregnancy, recovered by plasma exchange, that was followed by artificial abortion. Six months after the abortion, she no longer required plasma infusion every 3∼4 weeks for a relapse of TTP manifested thrombocytopenia, and continued complete remission for the next 6 months.
    In her second pregnancy, she had a immediate relapse of TTP and responded to plasma infusion until the 24th week. But, the TTP gradually became resistant to plasma infusion, and developed into toxemia with edema, hypertention and proteinuria in the 27th week. Although the TTP was alleviated by infusion of large amounts of plasma, the placental function changed irreversibly with white infarction, without response to plasma exchange. She delivered a 948 g live baby by cesarean section in 33th week. The baby had transient thrombocytopenia induced by platelet associated antibodies from the mother, but did not suffer from TTP.
    The pregnancy might be one of the causative and worsening factors of TTP, and the toxemia with numerous placental infarcts might be developed by recurring TTP in this patient.
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  • Hisami YAMAO, Tomomitsu HOTTA, Hidehiko SAITO
    1986 Volume 27 Issue 10 Pages 1938-1943
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    A transient remission of 2 months duration without any chemotherapeutic agents was attained in an 18-year-old female with acute hypoplastic myeloblastic leukemia. The remission was apparently associated with the blood transfusions. Normal hemopoiesis was convinced by normal CFU-C colony growth, the ferrokinetic study and normal karyotype of the bone marrow cells.
    Reports of spontaneous remission of acute myelogenous leukemia in adults have become increasing rare, but still present. Despite the association between spontaneous remissions and the severe infections in the literature, attempts to induce remissions with infectious agents or post-endotoxin sera have been unsuccessful. It is rather suggested that transient spontaneous remissions depend on uncommon nature of atypical leukemias.
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  • Mariko UEHARA, Mitsuhiro KIDA, Tomiko RYU, Shigetaka KONNO, Masahide K ...
    1986 Volume 27 Issue 10 Pages 1944-1949
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    A 64-year-old male was diagnosed as erythroleukemia in January, 1985. Abnormal chromosomes observed in his bone marrow cells were del (1)(p22), del(3)(p14p25), 2t(1;21)(p22;p11), 3del(7)(q22), dicentric (dic), various-sized rings and minutes, -5, -20, PCD (premature centromere division), endoreduplication, 4n etc. The proportion of cells with copies of t(1;21) and del(7)(q) were high when erythroblast cells increased. After DCMP-therapy, althogh the proportion of blast cells were decreased, those with abnormal karyotypes were not so much decreased. Among cells with abnormal karyotypes those with dic were decreased and those with rings and minutes were increased. Dic may have been changed to rings and minutes due to the effect of chemotherapy.
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  • Masao HIROSE, Hisako TOMIMOTO, Yoshifumi KAWANO, Kimiyo KAMADA, Tsuneo ...
    1986 Volume 27 Issue 10 Pages 1950-1956
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    A female of 1 month old was admitted on November 19, 1984 to Tokushima University Hospital because of petechiae, papular eruption and hepato-splenomegalia. The leucocyte count of peripheral blood on admittion was 355×103/μl with 91% of blasts.
    The diagnosis of acute monocytic leukemia was made from positive reaction of her leukemic cells for myeloperoxydase and α-naphtyle butylate esterase stain with NaF inhibition, and positive reaction of several monoclonal antibodies to monocyte antigen.
    A initial combination chemotherapy, neo-mini COAAP, was started with slight effect. But, the second treatment with VP-16 was so effective that the patient got into partial remission.
    But, spinal tap revealed the presence of CNS leukemia whose cells consisted of blasts and polymorphonuclear like cells in various differentiation stage, which were weakly positive for myeloperoxydase and positive for α-naphtyl butylate esterase stain. From these facts, it was suspected that monocytic leukemia cells which invaded into cerebro-spinal fluid developed lineage switch and maturation to polymorphonuclear like cells. This type of lineage switch and maturation from monoblast to polymorphonuclear like cell has not been known both clinically and experimentally.
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  • Hiroshi FUJII, Taira MAEKAWA, Hiroshi KAMEZAKI, Hitoshi OHNO, Kazuhiro ...
    1986 Volume 27 Issue 10 Pages 1957-1963
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    A 27-year-old Japanese female with 11 weeks' gestation was admitted because of progressive swelling of right cervical tumor in May, 1984. Her hematovrit was 39%; WBC, 7300/cmm with a normal differential counts; LDH, 1,300 mU/ml. The right cervical tumor was fist-sized and demonstrated to be thyroid origin by isotope scans, ultrasound tomography and computed tomography. In addition, stomach, colon, abdomen, liver and cervical lymph nodes were involved on admission. Microscopically, cervical lymph node was composed of sheets of packed undifferentiated lymphocytes. The cytoplasm of these cells was strongly positive with methyl-green-pyronin staining but negative with periodic acid-Schiff staining. Foaming histiocytes containing nuclear debris interspersed among the tumor cells disclosed the typical “starry sky” pattern. Surface phenotype of tumor cells had monoclonal B cell nature (μ, κ). The cells were also positive with OKIal and B1 monoclonal antibodies and negative with EBNA. Therefore, the diagnosis of Burkitt's lymphoma was made.
    After VEPA therapy, complete remission was obtained in July, 1984. A rapid relapse with signs of CNS involvement has been occurred in August. Skull irradiation and intrathecal injections of methotrexate and cytosine arabinoside could induce complete remission one month later. In October, however, leukemic transformation was developed and she died of sepsis in December. Chromosome analysis from peripheral blood in the phase of leukemic transformation revealed t(8;14)(q24;p32) with additional abnormalities including dup(1p), i(3p) and 1q trisomy.
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  • Kumiko YAMADA, Noriaki HORIUCHI, Yasumi SHINTANI, Kazuhiko MASUDA, Sad ...
    1986 Volume 27 Issue 10 Pages 1964-1970
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    A 53-year-old female was admitted on June 11, 1984, because of general fatigue. Laboratory investigations disclosed thrombocytopenia and anemia. The leukocyte count was 4,000/μl including 29.5% immature eosinophils, and 2% blast cells. Dysplasia of granular distribution and nucleus were found in neutrophils and lymphocytes. The bone marrow biopsy showed a marked fibrosis. Surface marker analysis of the peripheral mononuclear cells showed an increase of CALLA+ cells. The chromosome abnormality (karyotype 45, XX, -4, -5, -13, -18, 19p+, +3mar) was found.
    Two months after the admission, erythema exsudativum multiforme with high fever developed. The leukocyte count suddenly increased to 15,000/μl with 25% blast cells and 45% immature eosinophils. The patient died of sepsis and DIC. The autopsy showed a widespread infiltration of blast cells and immature eosinophils into the bone marrow, liver, spleen and other organs.
    This case was considered to be an eosinophilic leukemia with myelofibrosis, myelodysplasia of hemopoietic cells of 4 series, and the terminal increase of the blast cells. It provides a clue to clarify the mechanism of proliferation and differentiation of stem cells in myelodysplastic syndrome and myeloproliferative disorders.
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  • Toshiaki OKA, Yutaka SUZUKI, Azuma HIRAMOTO, Hiroshi AZUMA, Akimasa OK ...
    1986 Volume 27 Issue 10 Pages 1971-1976
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    We reported a case of inherited protein C deficiency with chronic relapsing purpura fulminance in the newborn period. He developed ecchymotic areas on the soles of his feet within a few hours after birth and then purpura fulminance developed. A diagnosis of disseminated intravascular coagulation was made and treated with exchange transfusions and repeated transfusions of fresh frozen plasma. His plasma protein C level was 17%. Two to 3 days after every plasma infusion, purpura fulminance and DIC developed so he received the oral anticoagulant (coumarin) every day. He also received Factor IX concentrates rich in protein C twice a week.
    The patient has remained asymptomatic and free of complications untill the age of 4 while receiving these treatments.
    The plasma protein C level of his father and mother were 80% and 38% respectively. These data suggest that this infant has a heterozygous protein C deficiency. The severity of the clinical findings is presumably related to the degree of the protein C deficiency.
    This patient also developed histidinemia. We do not know if his histidinemia is related to the protein C deficiency.
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  • Osamu ASAI, Yasunobu KURAISHI, Tatsuo AOYAMA, Satoshi FUNAKOSHI, Masat ...
    1986 Volume 27 Issue 10 Pages 1977-1982
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    The patient was a 21-year-old female who had been diagnosed as acute myeloblastic leukemia in 1979. Complete remission was achieved, but she relapsed in May 1985, and was treated with daunorubicin and cytosine arabinoside. On day 12 of therapy, tender, edematous red plaque was appeared on the anterior chest, and on next day similar lesions involved the trunk, lip, anticubital fossa accompanied by fever. Histological examination of skin biopsy showed a marked infiltration of neutrophils in the dermis. A diagnosis of Sweet's syndrome was made, and skin lesions were improved rapidly with prednisolone.
    Recently, cases of Sweet's syndrome associated with internal malignancy, especially with leukemia, have been reported, and etiological relationships with these diseases are noted. We report our case of patient, and those reported in the literature.
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  • [in Japanese], [in Japanese]
    1986 Volume 27 Issue 10 Pages 1983
    Published: 1986
    Released on J-STAGE: February 02, 2009
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  • Shojiro IKEMATSU, Masao HADA, Kazuko KUROSO, Morio ARAI
    1986 Volume 27 Issue 10 Pages 1984-1991
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    New concept, “molecular markers”, is introduced into the field of blood coagulation and fibrinolysis. An approach to make the pathological states of DIC clear using laboratory tests for molecular markers must be useful on the diagnosis and treatment of DIC. In this paper, the detection of crosslinked fibrin degradation products (XL FDP) with monoclonal antibodies and of α2 plasmin inhibitor-plasmn complex (α2 PI·Plm complex) by ELISA with differential antibodies are described. Both XL FDP and α2 PI·Plm complex are markedly elevated in patients with DIC. Elevation of XL FDP in DIC suggests that the crosslinking reaction has occured prior to the fibrinolysis of non-crosslinked fibrin. α2 PI·Plm complex assay will be helpful to differentiate the increased consumption and the decreased production state. These tests for molecular markers will give us a lot of informations for not only the basic analysis of DIC but also the clinical practise.
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  • Yasuo MAGARI, Morio ITO, Takashi ITOGA
    1986 Volume 27 Issue 10 Pages 1992-2007
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    The clinical significance of the mesurements of fibrinogen (fbg)-derivatives and other parameters related to coagulation and fibrinolysis in the diagnosis of disseminated intravascular coagulation (DIC) was studied. One hundred six patients (41 with DIC and 65 with non-DIC diseases) and 341 healthy subjects were studied. In the DIC patients, the plasma levels of high molecular weight fibrin (HMW-fbn), soluble fibrin monomer complex (sfmc), fibrinopeptide A (FPA) and FDP family were significantly higher than in the healthy subjects. However, fbg concentration was not significantly different between these 2 groups. In the serial mesurements of various parameters in 8 DIC patients, the plasma levels of HMW-fbn, SFMC and FDP family were observed to increase progressively from the pre-DIC stage to the DIC stage, but fbg concentration, platelet counts, and the activities of prothrombin, antithrombin III, plasminogen and α2 plasmin inhibitor were progressively decreased. The most of pre-DIC patients (70% or more of the cases) showed the abnormal levels of SFMC, FPA, FDP-D, E1 E2 and FDP-E.
    In conclusion, for the early diagnosis of the pre-DIC and DIC, the serial measurements of the following parameters were considered to be especially important; SFMC or FPA as the indicator of hypercoagulability state, and FDP-D, E1 E2 or FDP-E as of hyperfibrinolytic state.
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  • Tamotsu MATSUDA, Makoto SAKAI
    1986 Volume 27 Issue 10 Pages 2008-2013
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    11 Cases of aortic aneurysm including 3 cases of dissecting aneurysm and 8 cases of cardiac aneurysm, in whom typical consumption coagulopathy was observed, were presented. Mean age (±SD) of these was 79.8±11.0. In these cases, platelet counts and levels of fibrinogen, plasminogen and antithrombin III in plasma decreased, concentration of FDP in plasma increased, and prothrombin times prolonged. Euglobulin lysis times of these patients varied a great deal. The courses of these cases were generally long and abnormal hemostatic findings frequently improved following the administration of small doses of heparin (less than 10,000 units per day). Marked hemorrhagic symptoms were observed in only a few case, notwithstanding the fact that obivious consumption coagulopathy was present. Clinically, thrombotic symptoms, for example cerebral and/or renal infarction were more important. In a case of left cardiac aneurysm, multiple infarctions of the lungs developed. 4 cases among 11 ones of aortic aneurysm, complicated with local DIC, died of rupture of the aneurysm. In general, oral anticoagulant therapy was not effective in these patients, however an improvement of coagulation findings followin the administration of warfarin was observed in a patient of cardiac aneurysm.
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  • Yoichi SAKATA
    1986 Volume 27 Issue 10 Pages 2014-2021
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    Disseminated intravascular coagulation (DIC) is a pathological syndrome resulting from formation of thrombin and consumption of coagulation factors and platelets with associated activation of secondary fibrinolysis, encountered in numerous underlying diseases with varying degrees of severeity. The differences of manifestations are dependent upon the rate, the amount and continuance of thrombin formation as well as the level of functioning of the fibrinolytic system, liver, bone marrow, and reticuloendothelial system.
    DIC has been tried to be classified into many types, based on the pattern of laboratory data and on somewhat theoretical explanations.
    But right now, I think it is simple and best to classify DIC into acute and chronic type accoring to the clinical conditions and the severity of the laboratory manifestation.
    Cooper et al defined compensated DIC, in which most of the criteria for DIC are met but one or two of the usually depressed constituents are normal.
    Observing the cases of chronic DIC, we can say that there may be compensatory mechanism in regulation of coagulation and fibrinolytic system.
    However, addition of this new philosophical definition may make us confused in diagnosis and treatment of DIC. We had better clarify the meaning of compensation by the more scientific language.
    I tried to analyze the regulation of the number of platelets in DIC patients by observing the megakaryocytic colony formation according to the method of Messner. Fibrinogen and fibrin degradation products encountered in the patients who had ascites or pleural fluid with conformative DIC according to other laboratory data and clinical conditions and without were also tried to be evaluated.
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  • Norio KOBAYASHI, Hiroyuki SAITO, Hidemi GONMORI, Kiyoshi ANDO, Masashi ...
    1986 Volume 27 Issue 10 Pages 2022-2027
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    Hemostatic abnormalities of patients with various types of liver disease registered to the Japanese Research Committee on Coagulopathy including DIC were analysed. In patients with liver cirrhosis, apparent abnormalities were observed in various coagulation tests, which were scored 3 points per person on an average by a diagnostic criteia for DIC proposed by the Japanese Research committee on DIC. Since catabolism of both AT III and prothrombin labeled with radiolodine was not accelerated in these patients, decreased production of various clotting factors seemed to be a main cause of these abnormalities. In patients with acute or chronic hepatitis without severe hepatic dysfunctions, abnormality was minimal in various coagulation tests which were scored less than 1 point. On the basis of these findings, a modified diagnostic criteria for DIC have been proposed in fig 5.
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  • Hidenori KATOH, Keiichi OHSATO
    1986 Volume 27 Issue 10 Pages 2028-2034
    Published: 1986
    Released on J-STAGE: February 02, 2009
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    The most usual association of multiple organ failure (MOF) is with sepsis. And sepsis in one of the important primary disease of disseminated intravascular coagulation (DIC). Fourteen patients with DIC in sepsis and 13 patients with DIC in cancer were found during past 15 years in our institution. A comparative study of both groups was retrospectively done on their clinicopathological records. The characteristic findings obtained from the septic patients compared to the patients with cancer were as follows: 1) frequent association of shock (p<0.005) and organ failure (p<0.05), and 2) a decrease in components of complement such as C4 (p<0.05) and C3 (p<0.05). There was no difference in DIC score and the number of patients with microthrombi in vital organs at autopsy.
    Making a hemostatic disorder of mild course of DIC in animal experiment using dog, endotoxin induced a significant decrease in C3 and a development of marked metabolic acidosis followed by an increase and a development of marked metabolic acidosis followed by an increase in GOT, GPT, BUN and creatinine in serum. On the other hand, thromboplastin did not induce these changes, though coagulation and fibrinolytic abnormalities occurred similar to those in dogs administered endotoxin.
    Therefore, the organ failure found in DIC may not be always due to mechanical occlusion of microcirculation caused by fibrin thrombi. The activation of complement by endotoxin may produce biologically active substances which collaborate with a direct toxicity of endotoxin on cells and might result severe cellular damage of vital organs.
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