臨床血液
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
20 巻, 8 号
選択された号の論文の17件中1~17を表示しています
第20回総会
特別講演
臨床研究
  • 武田 成彰, 能塚 洋子, 加藤 秀典, 高木 輝, 能塚 洋子, 大里 敬一
    1979 年 20 巻 8 号 p. 879-885
    発行日: 1979年
    公開日: 2009/01/19
    ジャーナル 認証あり
    Euglobulin clot lysis time (ELT) has been said to be one of the most reliable methods for measuring fibrinolytic activity. Recently, Cl-Inactivator (Cl-INH) was identified as the only potent plasmin inhibitor in the euglobulin fraction, the presence of which had been neglected before. It is known that Cl-INH inhibits enzymes of the complement, kinin forming, coagulation and fibrinolytic systems.
    The present studies were undertaken to evaluate the inhibitory activity of Cl-INH on the ELT. Assay of 201 clinical cases revealed significant positive correlation between the concentration of Cl-INH in the euglobulin fraction and ELT. Furthermore, the concentration of this inhibitor increased in various diseases.
    In experimental studies, the effects of Cl-INH on ELT and kaolin activated ELT were investigated using the Cl-INH deficient plasma prepared by the method of antibody neutralization from the normal pooled plasma and factor XII deficient plasma. Lineality of dose-response curve was obtained between the Cl-INH and ELT as well as kaolin activated ELT. It was suggested that the Cl-INH influences ELT as an inhibitor of both plasmin and factor XIIa.
  • 堀田 知光, 平林 憲之, 内海 真, 滝田 資也, 山田 英雄
    1979 年 20 巻 8 号 p. 886-891
    発行日: 1979年
    公開日: 2009/01/19
    ジャーナル 認証あり
    The characteristics of back-ground factors and hematological recovery patterns in 13 patients with fully remitted acquired aplastic anemia were analysed. Nine out of 13 patients were suggested to have somewhat etiologic factors: chloramphenicol, gold colloid, organic solvent or hepatitis. There were no remarkable differences in the age and sex between these patients and 92 control patients with aplastic anemia. The initial hematological damages in these 13 patients were estimated mild in 5, moderate in 7 and severe in 1.
    The first signs of recovery in the hematological findings were noted from several months to one year, and remission was completed between 1 and 2 years after the onset of symptoms, and no reccurence of anemia has been observed until present time. The hematological recovery patterns showed no significant difference between the patients with or without androgen and/or crtico steroid therapy.
    The high incidence of secondary cases and the similar recovering patterns of hematological findings in these 13 patients suggest that common pathogenesis and recovering mechanism may influence to this type of aplastic anemia, and that their hemopoietic recovery may be mainly resulted from their own hemopoietic abilities rather than therapeutic effect of the drugs.
  • —本邦報告例(32例)の検討—
    山本 富一, 大熊 稔, 右京 成夫, 内野 治人
    1979 年 20 巻 8 号 p. 892-900
    発行日: 1979年
    公開日: 2009/01/19
    ジャーナル 認証あり
    Review of literatures on 32 cases of primary thrombocythemia including the present authors' case was made.
    1) Sex incidence of the disease was 14 and 18 in male and female, respectively.
    2) As to age incidence, it was found that more than half cases reviewed was over 60 years of age.
    3) Main symptoms of the disease were roughly divided into two: (1) symptoms due to bleeding of various origin (68.7%) and (2) symptoms probably attributable to changes of vascular obliteration (50%).
    Cases with swelling of the liver, spleen or lymph nodes were found in 41.9%, 46.8% or 9%, respectively.
    4) Hematologically, 90% of the cases had an increased peripheral platelet count of more than 100×104/μL, but there was no correlation observed between the peripheral platelet count and the bone marrow megakaryocyte or nucleated cell count. Furthermore, no definite tendency was observed in the results of coagulation tests.
    5) Finally, while chemotherapy with busulfan has been most widely adopted in treating primary thrombocythemia, it should be remembered that 6 cases of the disease has followed a relatively good course irrespective of no special therapy given.
症例
  • —I. 測定方法の検討—
    鈴木 節子, 松井 則明
    1979 年 20 巻 8 号 p. 901-909
    発行日: 1979年
    公開日: 2009/01/19
    ジャーナル 認証あり
    By a sensitive and specific method for detection of subclinical intravascular fibrin formation high molecular weight fibrinogen and fibrin complex (HMWFC) in plasma from patients through gel chromatography was determined, which was reported by Fletcher and co-workers. The authors devised a method for quantitative analysis of HMWFC in plasma.
    The specificity, stability and sensitivity of HMWFC in plasma was examined by this method and compared it with serial dilution protamin sulfate test, ethanol gel test and fibrin degradation products. Gel chromatography was performed essentially according to the method of Fletcher and co-workers. One milliter of plasma was applied to the acrylresin column (15×300mm) with Bio-gel A-15m and elution in borate buffer (0.02M, pH 7.5) was performed at a constant flow rate of 0.33ml per minits. The fractions of void portion was recognized by an internal marker of blue dextran. Fibrinogen in the four effluent fractions of void portion was determined by staphylococcal clumping test.
    Results were expressed as the concentration of HMWFC-binding-fibrinogen. The sensitivity of this assay was 0.07 μg/ml. This sensitivity was about 500 times as high compared with serial dilution protamin sulphate test or ethanol gel test. The plasma concentrations of HMWFC in normal control subjects were less then 0.56μg/ml. The plasma concentration of HMWFC in normal subject increased after addition of thrombin in vitro.
    From these results, it was concluded that the quantitative measurment of plasma HMWFC by this method was very useful for detection of intravasculer fibrin formation.
  • 丸尾 國造, 吉川 治哉
    1979 年 20 巻 8 号 p. 910-917
    発行日: 1979年
    公開日: 2009/01/19
    ジャーナル 認証あり
    A 72 year-old male with hemorrhagic tendency was hospitalized on August 31, 1976. He noticed the swelling of lymphnodes on both sides of the neck one month ago. On admission, petechiae on the extremities and many swollen cervical lymphnodes were found. The blood study showed the picture of pancytopenia with RBC 299×104/cmm, Hb 9.8g/dl, WBC 2,500/cmm (neutrophils 3%, monocytes 44%), Plt. 1.7×104/cmm. Bone marrow aspirates revealed a maturation arrest in granulopoiesis and no decrease in megakaryocytes; the finding seemed to be compatible with idiopathic thrombocytopenic purpura (ITP). However, a diagnosis of Hodgkin's disease of lymphocyte predominance was made by the biopsy of lymph nodes. Besides, polyclonal gammopathy, high Ig E value (7,332ng/ml), positive anti-nuclear factor (ANF) and positive direct Coombs' test were observed. VENP-therapy resulted a satisfactory clinical effect. A complicated interstitial pneumonitis in the right lung improved gradually.
    The case presented particular findings in the bone marrow similar to that of ITP, positive ANF, positive direct Coombs' test and high IgE value. It was presumed that these findings should be due to abnormalities of T-cell system.
  • 大内 栄悦, 森 和夫, 野村 暢郎, 鈴木 宗三, 岡辺 治男, 細谷 雄太, 清治 邦夫
    1979 年 20 巻 8 号 p. 918-927
    発行日: 1979年
    公開日: 2009/01/19
    ジャーナル 認証あり
    Two cases of systemic lupus erythematosus (SLE) accompanied by thrombotic thrombocytopenic purpura (TTP) were reported. Case 1 was 18-year-old girl. She was diagnosed as SLE because of Raynaud's phenomen, neuropsychiatric manifestation, LE cell, thrombocytopenia and hemolytic anemia. She died after the sudden onset of bleeding tendency, jaundice, convulsion and unconsciousness. Postmortam examination revealed that there were diffuse microthrombotic changes of small vessels in various organs and onino-skin like lesions in spleen and wire-loop like lesions in glomerulus. Case 2 was 38-year-old house wife. She was diagnosed as SLE because of Raynaud's phenomen, arthralgia, cellular cast, LE cell, pleuritis, alopetia and facial erythema. Suddenly, she lost consciousness, showing hemorrhagic tendency and became subicteric. Coagulation studies revealed decreased thrombocyte and fibrinogen, increased FDP, prolonged STT and K-PTT. She was recovered from unconsciousness by successful heparin treatment.
    These two cases of SLE were considered to be accompanied by TTP syndrome during their course. The possible relationship between SLE, TTP and DIC was discussed.
  • 滝内 鳩子, 巽 英二, 白川 茂, 内野 治人
    1979 年 20 巻 8 号 p. 928-932
    発行日: 1979年
    公開日: 2009/01/19
    ジャーナル 認証あり
    Two cases of Hodgkin's disease with pancytopenia were observed in our Department.
    Case 1. A 52-year-old male was admitted because of general lymphadenopathy, tonsillar swelling and fever. A biopsy was compatible with Hodgkin's disease, mixed cellularity type, and chemotherapy was begun. In spite of symptomatic improvement, pancytopenia developed gradually, and bleeding tendency appeared. He died four months after admission.
    Case 2. A 45-year-old female presented with fever, general lassitude, and easy bruising. Hematological examinations revealed moderate pancytopenia and hypoplastic marrow without abnormal cells. No remarkable lymph node enlargement was noted and she was treated as aplastic anemia. However, the patient did not respond well to treatment and pancytopenia developed. She died eight months after admission. At autopsy, deep lymph nodes were swollen and a diagnosis of Hodgkin's disease, lymphocytic depleted type, was made microscopically.
    Hematological abnormalities, including autoimmune hemolytic anemia and autoimmune thrombocytopenic purpura, occasionally have been reported. However, pancytopenia a sa major manifestation seems to be very rare and worth reporting, although its mechanism is unclear.
  • 木本 元治, 守永 真一, 山口 潜, 浅井 一太郎, 塚田 理康, 野沢 靖美, 原 満, 神山 隆一
    1979 年 20 巻 8 号 p. 933-942
    発行日: 1979年
    公開日: 2009/01/19
    ジャーナル 認証あり
    Five cases of (angio) immunoblastic lymphadenopathy were reported: A diagnosis of immunoblastic lymphadenopathy (IBL, Lukes) was made in two of them and that of angioimmunoblastic lymphadenopathy with dysproteinemia (AILD, Frizzera) in three of them from the biopsy findings of the lymph nodes.
    As signs and symptoms fever was noticed in four, drug hypersensitivity in two, lymphadenopathy in all (general in four and localized in one), hepatomegaly in three, splenomegaly in four, hypergammaglobulinemia in four and positive Coombs' test in two. The examination of the erythrocyte sedimentation rate revealed moderate to marked acceleration. C-reactive protein was from 2+ to 6+. serum lactic dehydrogenase was normal or moderately high, and PPDs was negative in four cases and weakly positive in one.
    Treatment with MOPP schedule in two cases resulted a good response in one. Death was due to various sorts of infection in four of five cases and autopsy was done in three.
    Duration of the disease from the first visit was between 11 and 27 months. Lymphocyte subpopulation studies of the lymph node biopsy materials from the three cases revealed a proliferation of IgA monoclonal B-cells by SIg in a case of IBL and a proliferation of polyclonal B-cells in a case of AILD; in another case of AILD T- and B-cells were within normal limits.
  • 白倉 卓夫, 村井 善郎, 高橋 竜太郎, 井上 達
    1979 年 20 巻 8 号 p. 943-951
    発行日: 1979年
    公開日: 2009/01/19
    ジャーナル 認証あり
    A case of chronic neutrophilic leukemia (CNL), associated with polycythemia later, was presented. Patient, a 75-year-old Japanese woman, was admitted because of the generalized lymphadenopathy, hepatosplenomegaly (one and three fingerbreadths below the respective costal margin) and leucocytosis (26,000/μl) in October, 1974. On admission, the laboratory study revealed a slight anemia and WBC count of 19,400/μl (1.0% myelocyte, 1.0% metamyelocyte, 25.5% band-formed, 63.0% segmented neutrophil, 1.0% monocyte and 8.5% lymphocyte). LAP score was within the normal range, and no Ph1 chromosome was observed on chromosomal analysis. Bone marrow finding was a hypercellularity with the myeloid cells showing various stages of maturation. There was neither eosinophilia nor basophilia. Thereafter, neutrophils gradually increased and reached 100,000/μl. Furthermore, polycythemia (RBC mass of 38.5 ml/kg) without an elevation of erythropoietin was first detected to coexist in October, 1975. Thus, the treatment with pipobroman was instituted. However, a rapid increase reaching 178,560/μl in neutrophils occurred in April, 1978, and she died three days later. Postmortem examination revealed an infiltration of mature neutrophils in the splenic sinusoids and red pulp, where necrotic foci were observed. Contrary to these findings, such bone marrows as vertebral, sternal and femoral were replaced by immature myeloid cells, but mature neutrophils were partly observed in these bone marrows. Therefore, it is suggested from these findings that the case here presented might be a type of CNL terminating in blastic crisis.
  • 横谷 進, 須田 年生, 柳沢 正義, 溝口 秀昭, 三浦 恭定
    1979 年 20 巻 8 号 p. 952-957
    発行日: 1979年
    公開日: 2009/01/19
    ジャーナル 認証あり
    A 13-year-old girl was admitted to our hospital for the purpose of examining the cause of anemia. She had been pale for about one year, but there had been no limitations in her daily life. Hematological examinations revealed pancytopenia and cellular bone marrow with increase in erythroblasts and decrease in both megakaryocytes and granulocytes. Sugar water test and acidified serum test were positive, but repeated urinalyses failed to show hemoglobinuria or hemosiderinuria. Leukocyte alkaline phosphatase score was normal or occasionally elevated. From these findings, she was diagnosed as having aplastic anemia-PNH syndrome in a narrow sense.
    Bone marrow cells were cultured in a plasma clot or in soft agar in order to investigate changes in erythrocytic and granulocytic precursor cells. Both erythrocytic and granulocytic colony formation was markedly decreased. These findings may indicate that abnormalities of hemopoietic stem cells occurred in this case.
    Moreover, 18 reported cases with PNH below 20 years old were reviewed. Two cases among them had neither hemoglobinuria nor hemosiderinuria.
  • 長谷川 恵子, 池田 整昭, 浜本 邦洋, 緒方 博子
    1979 年 20 巻 8 号 p. 958-964
    発行日: 1979年
    公開日: 2009/01/19
    ジャーナル 認証あり
    A case of 6 5/12 year-old girl with β-thalassemia intermedia was reported.
    She had so-called ”Cooley face”, severe anemia, jaundice and hepato-splenomegaly. Laboratory examination revealed microcytic anemia (Hb 4-5 g/dl) associated with erythroblastosis and poikilocytosis, shortened erythrocyte life span (51Cr T 1/2 was 4 days), hypersideremia, ineffective erythropoiesis and skeletal changes.
    Hemoglobin F and Hemoglobin A2 were significantly elevated. α/β synthetic ratio of globin in circulating reticulocytes was 1.73.
    She was diagnosed as being heterozygote for β-thalassemia gene, and so-called ”thalassemia intermedia” was appropriate term for this case, because of severe symptoms associated with hypersplenism.
  • 高橋 芳右, 塚田 恒安
    1979 年 20 巻 8 号 p. 965-977
    発行日: 1979年
    公開日: 2009/01/19
    ジャーナル 認証あり
    A case of immune hemolytic anemia induced by triamterene, the first case in the world, was reported. A 54-year-old female was admitted to our institute because of abdominal pain, lumbago, cyanosis and shock after one month treatment with Daiteren-S (a compound of triamterene and chlorphenamide). On admission she revealed remarkable cyanosis in the extremities, lips and trunk, and they disappeared when she was warmed. Her laboratory findings showed mild anemia, reticulocytosis, elevations of lactic dehydrogenase, bilirubin and serum iron, and low haptoglobin level. There were also remarkable hemoglobinuria and hemoglobinemia which were complicated with acute renal failure.
    A direct antiglobulin test done with a polyvalent reagent was positive. The patient's serum caused agglutination of normal red cells in the presence of triamterene and caused an increase of the partial hemolysis of both trypsin-treated erythrocytes and red blood cells taken from a patient with paroxysmal nocturnal hemoglobinuria in the presence of complement. Its optimal temperature was between 20° to 37°C. The minimal concentration of triamterene was about 5 ng/ml. From the results of antibody-neutralization test and treatment with 2-mercaptoethanol, IgM antibody with λ light chain could be demonstrated. The triamterene seemed to bind strongly to the red blood cells in vitro but in vivo there was no adsorption to red cells. Haptenic inhibition was not demonstrated.
    From these results, in this case, it was assumed that IgM (λ) antibody against triamterene was generated, agglutination was observed when patient's serum, triamterene and red cells were incubated together, and this antibody was capable of activating complement and caused acute intravascular hemolysis by immune complex mechanism. The antibody was found to cross-react with methotrexate which has a structure similar to that of triamterene.
  • 二宮 規郎, 森 秀樹, 鈴木 とし子, 武田 章臣, 都 通彦, 内藤 真, 高橋 潔
    1979 年 20 巻 8 号 p. 978-986
    発行日: 1979年
    公開日: 2009/01/19
    ジャーナル 認証あり
    This patient was a 8 year and 8 month-old girl. She had a diagnosis of β-thalassemia which was established from hematological examination and hemoglobin study.
    Peculiar storage cells found in the bone marrow aspirates were examined light microscopically, histochemically and electron microscopically. Light microscopically, most of the storage cells had a relatively abundant, pale cytoplasm with faintly recognizable striations in May-Giemsa stain, showing a miniature of Gaucher cells. Electron microscopically, the most striking feature of the Gaucher-like cells was the presence of numerous intracytoplasmic variable-shaped inclusions. Their inclusions were filled with fibrillar materials. Though tubular structures were often intermingled, they were usually narrow as compared to those of Gaucher cells. Enzyme cytochemically, acid phosphatase activity was proved in or around the storage inclusions, suggesting their lysosomal origin. Histochemically, it might be suggested that glycoproteins were the major component of the Gaucher-like cells. Possible mechanism of storage in the Gaucher-like cells has been discussed in this disorders.
    This is probably the first case of thalassemia with Gaucher-like cells in the bone marrow in Japan.
  • 小山 隆三, 石川 邦嗣, 小関 純一, 中野 良昭, 後藤 幹雄
    1979 年 20 巻 8 号 p. 987-993
    発行日: 1979年
    公開日: 2009/01/19
    ジャーナル 認証あり
    Recently we encountered a rare case of acute promyelocytic leukemia which showed interesting cytochemical findings. The patient was a 40-year-old male. His chief complaints were anemia and gingival bleeding and he was admitted to our clinic on April 10, 1976. The peripheral blood findings on admission revealed severe anemia and slight leukopenia.
    Blood examination showed 50% of atypical promyelocytoid cells with folded, indented and dumbell-shaped nuclei. In addition these atypical cells showed abundant azurophilic granules and multiple Auer rods.
    A sternal aspirate revealed hypercellularity with a diffuse proliferation of atypical promyelocytoid cells. The plasma fibrinogen was 160 mg/dl. The diagnosis based on the peripheral blood and bone marrow findings was acute promyelocytic leukemia. As a result of cytochemical analysis, these leukemic cells showed a strong positive peroxidase, betaglucuronidase, acid phosphatase and naphthol-AS-D-chloroacetate esterase activity. On the other hand, these leukemic cells showed a strong alpha-naphthyl acetate esterase activity on cytochemistry and polyacrylamide gel electrophoresis of leukemic cell extracts. Moreover this enzyme activity was remarkably inhibited by sodium fluoride.
    Further, electronmicroscopic DAB (diaminobenzidine) reaction showed peroxidatic catalase activity in granules on the alkaline side. These cytochemical findings suggested that leukemic cells of the case presented here involved a differentiation into both granulocytic and monocytic series, the so-called dysdifferentiation, in a neoplastic process.
  • 吉川 治哉, 丸尾 國造
    1979 年 20 巻 8 号 p. 994-999
    発行日: 1979年
    公開日: 2009/01/19
    ジャーナル 認証あり
    In three cases of acute lymphocytic leukemia (ALL; 67 years old male, 14 female and 31 male), diffuse interstitial pneumonia developed during the period of treatment with large dosis antibiotic which was given for fever in the granulocytopenic stage after the antileukemic chemotherapy. Immediately, treatment with sulfamethoxazole-trimethoprim (ST) started; two tablets three times daily. The responce was rapid and repeated throat cultures resulted negative.
    So far in our clinic, diffuse interstitial pneumonia has rerely been seen in cases of acute non-lymphocytic leukemia (ANLL) inspite of their frequent incidences, while ALL has sometimes combined with pneumonia. As regards the steroid, a large dosis was intermittently given in cases of ANLL as remission induction chemotherapy, while a rather small dosis for a long period in those of ALL. It was presumed that a long-term treatment with prednisolone in the ALL may be one of the factors for frequent occurrence of diffuse interstitial pneumonia.
    It was suggested that ST administration should be one of the adequate supportive therapy when the X-ray examination showed a finding of diffuse interstitial pneumonia for patients with acute leukemia.
例会
feedback
Top