Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
Volume 21, Issue 2
Displaying 1-19 of 19 articles from this issue
  • Fumimaro TAKAKU
    1980 Volume 21 Issue 2 Pages 125-135
    Published: 1980
    Released on J-STAGE: January 26, 2009
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    Alterations in bone marrow CFU-C and CFU-E in various hematological disorders were studied to clarify the pathogenesis of these disorders from the point of view of hematopoietic stem cells. Emphasis was put on a series of anemia caused by the disturbance of red cell production, polycythemia and leukemias. In aplastic anemias. CFU-C and CFU-E were decreased invariably and the presence of mononuclear cells which are suppressive both to CFU-C and CFU-E was demonstrated in peripheral blood or bone marrow of the patients. It was demonstrated that monocytes and macrophages were playing an important role in this suppression. In sideroblastic anemia, presence of 2 clones of stem cells, the one normal clone forming normal erythroblast colony and the other abnormal clone which develops to the ringed sideroblast in vitro and can not form the erythroblast colony, was demonstrated. By cultivating the peripheral blood of a patient with acute non-lymphocytic leukemia, we could make a leukemia blast colony in vitro. This leukemia colony was consisted of leukemic blasts and the kinetic study on leukemia colony formation showed that each colony was developed from each leukemic stem cell. In chronic myelogenous leukemia, both CFU-C and CFU-E were increased in numbers. CFU-E in chronic myelogenous leukemia was dependent on the erythropoietin.
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  • Pyong-young KIM, Junko ETO, Takeo FUJIMOTO, Yoshiaki SASAKI, Yasuhiko ...
    1980 Volume 21 Issue 2 Pages 136-143
    Published: 1980
    Released on J-STAGE: January 26, 2009
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    On the basis that circulating L-asparagine can be reduced to negligible level within minutes of administration of therapeutic doses of L-asparaginase, a two dosage theraputic regimen of enzyme was investigated to determine the effectiveness and toxicity of even lower doses of the enzyme in pediatric patients with advanced leukemia.
    Schedule A consisted of vincristine, 2mg/M2/week×4, I. V., prednisone, 60mg/M2/day×21, P. O., and L-asparaginase 300IU/M2/day×15, I. V. Schedule B consisted of vincristine and prednisone, same as schedule A and L-asparaginase 600IU/M2/day×15, I. V.
    Among 26 patients, 13 were randomized to schedule A, and 13, schedule B.
    Marrow remissions were induced in 10 (83.3%) of 12 patients with schedule A, in 8 (66.7%) of 12 with schedule B. The over-all response rate for the study was 18(75%) of 24.
    No death contributed to both regimens was observed. Among the serious side effects of this enzyme, such as hypersensitivity reactions, hyperglycemia, pancreatitis, and seizure, only one patient in schedule A, developed reaction comprising urticaria.
    The other signs of toxicity of low plasma fibrinogen levels, abnormal liver function tests, hypoproteinemia, hypolipidemia and leukopenia were observed in most instances to even lower doses of the enzyme. But these were mild and reversible.
    These data indicated much lower doses of the enzyme are effective in the therapy of acute leukemia, and seemed to lessen the serious toxicity.
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  • Toshiko MOTOJI, Manabu MUSASHI, Riichi KURANE, Kyoichi TOTSUKA, Junich ...
    1980 Volume 21 Issue 2 Pages 144-150
    Published: 1980
    Released on J-STAGE: January 26, 2009
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    Several factors were studied which might affect the prognosis of 55 patients with ANLL and ALL. Amoung our results, we found that the remission rate of patients over 50 years old was 36.0%, as compared to a remission rate of 85.0% for the patients under 50, suggesting that a patient's age seems to most strongly affect remission. Age also affected the length of survival, which was only 5.9 months for the over-50 group, compared to 18.8 months for younger patients. In ANLL, the remission rate was apparently higher for patients with low WBC (<3×104/ul) and with low peripheral blast cell counts (<2×104/ul) than for those with high WBC and high blast cell counts. In cases which showed low LAP activity (<190), the remission rate was significantly higher (70.0%) than those with high LAP activity (29.4%) (p>0.05). The median survival time also was longer in the low LAP activity group (p<0.005). The remission rate was higher and survival longer in patients in whom no chromosome abnormality was seen. Thus, our results suggest that an age under 50, a low peripheral leukocyte count, low LAP activity and an absence of chromosomal abnomalities are the factors pointing to the most favorable prognosis in an acute leukemic patient.
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  • Part 1. Collection of Neutrophils by Haemonetics Model 30
    Keiji FUKAZAWA, Hisato KIGASAWA, Hirokazu NISHIHIRA, Chiyomi SATO, Ats ...
    1980 Volume 21 Issue 2 Pages 151-159
    Published: 1980
    Released on J-STAGE: January 26, 2009
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    This study was conducted to improve the yield of neutrophils collected for transfusion from normal donors by means of the Haemonetics Model 30 Blood Cell Processor.
    A total of 78 leukaphereses have been performed on 67 donors. A mean yield of 13.9±7.5 (×109) neutrophils was obtained by the pretreatment of the donor with hydrocortisone (Solu-Cortef, 200 mg. i.v.) or dexamethasone (6 mg. p.o.) and the addition of high molecular hydroxyethyl starch (HES) to the extracorporeal circulation.
    The collection time of 3 minutes on each cycle seemed most adequate for the yield of neutrophils.
    High molecular weight HES was better than low molecular one in increasing the number of neutrophils harvested and in reducing the total fluid volume infused.
    Adverse effects were minimal. The structure and function of the processed neutrophils were not damaged or changed.
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  • Part 2. Clinical Use of Neutrophil Transfusion
    Keiji FUKAZAWA, Hisato KIGASAWA, Hirokazu NISHIHIRA, Chiyomi SATO, Ats ...
    1980 Volume 21 Issue 2 Pages 160-168
    Published: 1980
    Released on J-STAGE: January 26, 2009
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    Against the life threatening infections in severe neutropenic patients (neutrophils <500/μl), neutrophils obtained by Haemonetics Model 30 from normal donors, were transfused to 26 cases (21 patients) on 80 occasions. An average of 10.9±8.3 (×109)/m2 neutrophils was administered per transfusion on every other day until the clearing of infections (1∼7 transfusions, mean: 3.1).
    The mean of neutrophil increment was 322±347/μl.
    Definite clinical improvement was noted in 21 cases (80.8%). Mild or moderate degree transfusion reactions were observed on 12 occasions (15%). These reactions were lessoned by slowing the infusion rate and by administering antihistaminics. Based on neutrophil transfusions, intractable leukemia can now be induced into remission with agressive chemotherapy.
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  • Takeshi ISHIZAKI, Kazuko HASHIZUME, Masako KOMETANI, Toshie TANAKI, To ...
    1980 Volume 21 Issue 2 Pages 169-178
    Published: 1980
    Released on J-STAGE: January 26, 2009
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    In twenty-one patients with leukemia associated with DIC, levels of serum complement components (C1q, C1s, C3, C4, C3 anaphylatoxin, C5, C9 and factor B) were measured sequentially by single radial immunodiffusion method. The levels of C5, C9 and factor B on admission without DIC are significantly higher than those of normal control (p>0.05). The levels of C1q, C1s, C3 and C3 anaphylatoxin in DIC, which was diagnosed by high FDP (more than 20μg/ml) and low fibrinogen (less than 200 mg/dl) levels, are significantly lower than those of normal control (p<0.05). The lower the levels of C3, C4, C5 and factor B are, the worse the prognoses of patients—good parameters for prognosis. Therefore, it is of prognostic value to evaluate the levels of complement components especially C1q, C1s, C3 and C3 anaphylatoxin in DIC associated with leukemia.
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  • Hoyu TAKAHASHI, Kazuo KOIKE, Masakazu ITOH, Akira HATTORI, Isao KOBAYA ...
    1980 Volume 21 Issue 2 Pages 179-188
    Published: 1980
    Released on J-STAGE: January 26, 2009
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    The clinical features of 47 patients with von Willebrand's disease were studied. Nose bleeding was the most common symptom and ecchymoses, bleeding after tooth extraction, gingival bleeding and prolonged bleedings from trivial wounds were fairly common. Gynecological bleedings were also prominent symptoms in females. One patient revealed a joint bleeding with arthropathy and one patient showed an intramuscular bleeding. The mode of inheritance was mainly autosomal dominant, but there were five sporadic cases. The parental consanguinity was recongnized in three families.
    Von Willebrand factor activity (VIII R: WF) appeared to be the most useful parameter to diagnose von Willebrand's disease. There were five patients with undetectable factor VIII related antigen (VIII R: AG), but VIII R: AG and factor V III procoagulant activity (VIII: C) were within the normal range in a number of patients. An increased anodal migration of VIII R: AG was detected in 14 of 18 patients in which was performed two-dimensional crossed immunoelectophoresis. Among classical cases there were many patients who showed increased electrophoretic mobility of VIIIR: AG.
    There was a significant positive correlation between VIII R: AG and VIII: C (r=0.710, p<0.001) and the ratio of VIII: C/VIII R: AG was more than 1.0 in patients with decreased VIII R: AG. There was no significant correlation between VIII: C and VIIIR: WF. Although there was a positive correlation between VIII R: AG and VIII R: WF (r=0.407, p<0.02), there was a discrepancy between them in patients with normal VIII R: AG. This finding suggested that there was a qualitative abnormality of the factor VIII molecules in these patients.
    It was assumed that patients with von Willebrand's disease had a defect in primary hemostasis with reduced VIII R: WF due to a quantitative and/or qualitative abnormality of the factor VIII protein.
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  • Yasunari NAKATA, Akira KONDO, Mikio KATAOKA, Kenta TAKASUGI, Toshimasa ...
    1980 Volume 21 Issue 2 Pages 189-194
    Published: 1980
    Released on J-STAGE: January 26, 2009
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    An immunopathological study of 21 patients with thymoma was perfomed. The median age was 51, with range of 26-69 years. There were 11 male and 10 female. All thymoma of this series were detected by thymectomy, biopsy and necropsy. Myasthenia gravis was present in 10 patients, including 1 patient who also had pure red cell aplasia. Sjögren's syndrome was present in 2 cases, and pure red cell aplasia in 1. Four cases were predominantly epitherial cellular type, 9 were lymphocytic and 8 were mixed. The average of circulating lymphocyte counts of the thymoma patients was 2986/cmm and was higher than normal. There was an increase in the number of non-T and non-B lymphocyte (null-lymphocyte). Both counts of T and B-lymphocytes were within normal value. The response of the peripheral lymphocyte in thymoma patients in vitro against PHA-P stimulation was significantly suppressed than healthy lymphocyte. Antinuclear antibody was found in 75% serum of thymoma patients.
    In 9 thymoma obtained at thymectomy, the median percentage of T-lymphocyte was 72 and B-lymphocyt was 4. Sixteen patients were studied prospectively before and after thymectomy, and there was a decrease in peripheral lymphocytes after thymectomy that was statistically significant. The proportion of decreased lymphocytes after thymectomy was mainly null-lymphocytes. The thymoma patients had abnormality of humoral and cellular immunity.
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  • Tetsuo SUDA, Mitsuhiro OMINE, Jun TSUCHIYA, Tadashi MAEKAWA, Yoshitomo ...
    1980 Volume 21 Issue 2 Pages 195-199
    Published: 1980
    Released on J-STAGE: January 26, 2009
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    The purpose of this study is to determine the prophylactic effect of orally administered amphotericin B (AMPH-B) for the prevention of systemic fungal infections in patients with acute leukemia, malignant lymphoma or multiple myeloma. On admission, half of these were randomly assigned to receive oral as well as gurgle administration of AMPH-B in doses large enough to suppress overgrowth of fungus in the intestinal tract and mouth. The others received no prophylactic administrations.
    Although there was no difference in terms of the prevention of development of severe systemic fungal infections between the two groups, cultures obtained from the stool and saliva of patients who received AMPH-B showed significantly decreased positivity as compared with those of patient without AMPH-B.
    It is suggested that even if the patient becomes severely ill and impossible to take the drugs orally, the administration of AMPH-B should be continued in a possible way for the prevention of the development of sytemic fungal infection because the risk of the development is very high in these patients.
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  • Shoichi INOUE, Hiroshi NAGATA, Hirokazu YOZAWA, Tsugio TERAI, Hiroshi ...
    1980 Volume 21 Issue 2 Pages 200-207
    Published: 1980
    Released on J-STAGE: January 26, 2009
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    A 59-year-old man with multiple myeloma is reported. Examinations performed on admission revealed the presence of both IgG myeloma protein and Bence Jones protein of λ type in the serum, whereas no Bence Jones protein in the urine. This Bence Jones protein was a tetramer of λ type light chains physiochemically. Evidences of gelatinous bulky clots, impaired clot retraction and prolonged thrombin times were noted. Electron microscopic study revealed the disturbance of fibrin clot formation seemed to be due to the interference of the M protein with fibrin aggregation—α-α and γ-γ cross-linkages. Significant increase in the number of the T cells seemed to suggest the relation to the immaturity of the myeloma cells.
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  • Hirofumi NAGOYA, Hiromi NISHI, Kazuo KURA, Masanobu SAITO, Takeo KUROY ...
    1980 Volume 21 Issue 2 Pages 208-213
    Published: 1980
    Released on J-STAGE: January 26, 2009
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    A 14-year-old boy was admitted because of the intractable nasal bleeding. He had been in excellent health until the admission. More than three months before the admission, he had been given indomethacin, sulpyrine, aminopyrine, ampicillin and midecamycin because of common cold. About one and a half months bfeore admission, he complained of general fatigue, palpitation and shortness of breath and he was noticed the icteric skin and sclerae at the time of admission. Physical examination on admission revealed icteric skin and sclerae, hepatosplenomegaly and multiple petechiae on the lower extremities. Liver function tests showed intrahepatic obstructive jaundice. The GOT was 742mU/ml, GPT 900mU/ml, LDH 300mU/ml, total bilirubin 6.3mg/dl (direct 5.0, indirect 1.3) and LAP 541 IU. Hematological examination showed sever pancytopenia. The RBC count was 273×104/mm3, Hb 8.1g/dl, Ht 23%, MCV 83μ3, MCH 29.3 μg, MCHC 35.3%, respectively. The WBC count was 900/mm3 with a normal differential count except for the presence of monocytoid atypical lymphocytes (3%). The platelet count was 4000/mm3. The bone marrow preparation revealed a severely aplastic and fatty marrow associated with an infiltration of vacuolated macrophages and reticulum cells which was considered to be characteristic to the hepatitis-aplastic anemia syndrome. The significance of vacuolated macrophages remians still to be clarified. The HBs antibody had been detected during his hospital days. Therefore, the present case might be probably aplastic anemia following acute hepatitis B. He developed the culture-negative sepsis and died of the intracranial hemrrhage and gastrointestinal bleeding. The clinical course from the onset of hepatitis was 20 weeks and the autopsy was not permitted.
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  • Hideko TASAKA, Jun OKAMURA, Yoshiko IKUNO, Kazuo TAKAYAMA, Hiromi TERA ...
    1980 Volume 21 Issue 2 Pages 214-219
    Published: 1980
    Released on J-STAGE: January 26, 2009
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    A 7-year-old boy who was diagnosed as having acute lymphocytic leukemia in December 1974, had been treated with combined chemotherapy and cranial irradiation.
    After the sixth relapse, he became resistant to combined chemotherapy.
    He received drip infusion of bone marrow cells (5.7×108/kg) from the elder brother with identical HLA A, B and D antigens. Conditioning regimen consisted of cyclophosphamide and total body irradiation. During transplantation he was nursed in reverse isolation room. For prevention of graft-versus-host reaction (GVHR), he was given methorexate 15mg/M2 on the day of trasplantation followed by 10mg/M2 on 3, 5, 10th day and on weekly basis thereafter.
    Nadir of white blood cell count (200/mm3) was six days after transplantation. He had been maintained neutrophil count above 500/mm3 after the 19th day. Disappearance of blast cells in bone marrow was in the 5th week. After then he never received blood component transfusions. Grade II GVHR was noticed around the third week and subsided around the twelveth week.
    He developed the third generalized Herpes zoster infection in the twelveth week and sebsequently expired with heart failure due to lung edema sixteen weeks after transplantation.
    Necropsy revealed bone marrow remained free of disease although leukemic infiltration with tumor formation in the lungs, liver, kidneys, pancreas and testicles.
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  • Kunizo MARUO, Haruya YOSHIKAWA, Katsuro KUBOYAMA, Kiyoshi YOKOE, Tetsu ...
    1980 Volume 21 Issue 2 Pages 220-232
    Published: 1980
    Released on J-STAGE: January 26, 2009
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    Case 1: 55 years old female. Ig A-K type myeloma. Two courses of MP-therapy (Melphalan 10mg/d×7d and Prednisolone 30mg/d×7d) succeeded to give rise a hematological remission, although she was not able to walk owing to pain due to pseudoarthrosis of her hip joints. Endoprosthesis of the right femoral head made possible to walk by herself. In spite of repeated MP-therapies, a hematological relapse with extramedullary myeloma (EM) occured. Two courses of AAAP-therapy—ACNU 50mg/d IV (drip over 4 hrs), Adriamycin 20mg/d IV (push), Methotrexate 25mg/d IV (push), and Prednisolone 60mg/d IV (push): once a two or three weeks—was effective so that hematological remission and diminution of EM were observed.
    Case 2: 64 years old female. Bence Jones L type myeloma.
    A hematological remission was seen after one course of MP-therapy, however she was unable to walk because of pain due to pseudoarthrosis of the left hip joint. The endoprosthesis succeeded to enable her to walk on crutches. After that, a hematological relapse with EM occured in spite of repeated MP-therapies. Since the radiation with 60Co 6,600 rads failed to give any beneficial result, it was observed that AAAP-therapy resulted in a complete diminution of EM as well as a hematological remission.
    Thus, AAAP-therapy was disclosed one of the excellent chemotherapies for myeloma in relapse.
    And we considered that it would be worth doing endoprosthesis of the femoral head to enjoy a normal day of activity in a patient of myeloma with pain due to pseudoarthrosis of the hip joints.
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  • Hideo ENOKIHARA, Yasuko ISHIKAWA, Sakae HAZAMA, Kenji SAITO, Kouji HIR ...
    1980 Volume 21 Issue 2 Pages 233-239
    Published: 1980
    Released on J-STAGE: January 26, 2009
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    A 81-year-old man was admitted with a chief complaint of the ecchymoma after slight impact on the left upper extremity. Coagulation studies revealed moderate thrombocytopenia, decreased levels of fibrinogen, factor VIII and antithrombin III, an increase of FDPs, and prolongation of prothrombin time and activated PTT. The chest X-ray film and U.C.G. showed an aneurysm of the thoracic aorta. The surface scanning after intravenous 131I-fibrinogen demonstrated an increased accumulation of the radioactivity over the aneurysm. Extensive studies failed to disclose any malignant diseases.
    A diagnosis of consumption coagulopathy in association with the aneurysm was made. He was treated with intravenous heparin (20,000 units/day) with rapid improvement; platelet and fibrinogen increased, FDPs levels decreased. However, the conbination therapy with aspirin and warfarin was of little effect.
    The association of consumption coagulopathy with aneurysm seems to be relatively rare. The pathogenesis and treatment of this disease were discussed.
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  • Kijo DEURA, Zenichiro ISHII, Masanori SHIMOYAMA, Keisuke MINATO
    1980 Volume 21 Issue 2 Pages 240-250
    Published: 1980
    Released on J-STAGE: January 26, 2009
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    A 76 year-old man was admitted with complaints of nasal bleeding, Raynaud's symptome and dyspnea in the cold, which were related to serum hyperviscosity. Physical examination demonstrated neither lymphadenopathy nor hepatosplenomegaly, but large lymphoid cells were increased in the peripheral blood and bone marrow, most of those lymphoid cells carried the monoclonal IgM (λ) on the surface. The patient serum showed hyperproteinemia (8.2 g/dl) and positive Sia test. Pathological immunoglobulin was identified as IgM (λ). Bence Jones protein in urine was also demonstrated.
    Serum viscosity was as high as 111 times water at room temperature by simplified method using white blood cell pipette. Its viscosity was increased at 0∼4°C. Both pyroglobulin and cryoglobulin were demonstated in the serum, serum gelation was occured upon heating to 56°C over 60 minutes.
    When pyroglobulin and cryoglobulin were removed from the serum, the M component decreased of the resulting supernatant, but it was found in the cryoglobulin. Further examination showed that cryoglobulin consists of mixtuers of monoclonal IgM (λ) and IgG. Plasmapheresis of about 1,600 ml/week was effective in reducing the hyperviscosity syndrome.
    After 7 months from the onset, the patient died of pneumonia and autopsy was done. Autopsy showed that the cause of death was cytomegalovirus pneumonia. Diffuse lymphoplasmacytoid cell proliferation was in the bone marrow but not in the spleen, liver and lymph nodes. “Multiple lymphatic thrombosis” was demontrated, thought to be related to serum hyperviscosity.
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  • Masaharu OCHI, Shoji ARAKAWA, Shinjiro TODO, Masahiro MINO, Shinsaku I ...
    1980 Volume 21 Issue 2 Pages 251-258
    Published: 1980
    Released on J-STAGE: January 26, 2009
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    The adult type of chronic myelogenous leukemia (CML) is rare in childhood. A 8 year-old boy with Ph1 positive CML had been treated with 0.5∼1.5 mg daily dosis of busulfan and he had had a good remission for 45 months. At 12 years old, he developed a bone marrow hypoplasia which seemed to be induced by busulfan. This hypoplasia continued for 4 months, and after a while the blastic transformation occurred. In the blastic phase he was treated with a combination regimen of adriamycin, vincristine, cytosine arabinoside and prednisolone, but he did not have a remission. Three months after the onset of blastic transformation, he died of pneumocystis carinii pneumonia.
    We repeated the cytogenetic studies throughout the chronic, hypoplastic and blastic phases in this case. During the hypoplastic phase we expected the existence of Ph1 negative cells which had not been recognized throughout the chronic phase, but all cells were found to have the Ph1 chromosome. In association with blastic transformation we recognized the appearance of hyperdiploid cell lines. The process of this clonal evolution seemed to correspond to the resistance to therapy for the blastic phase.
    The clinical significance of the busulfan induced hypoplasia and chromosome findings in blastic phase were discussed.
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  • Atsushi TOGAWA, Norio SASAKI, Masao URABE, Shyozo CHIBA, Yukio IMAMURA ...
    1980 Volume 21 Issue 2 Pages 259-265
    Published: 1980
    Released on J-STAGE: January 26, 2009
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    A 25-year-old female with acute promyelocytic leukemia and tetraploid blast cells is reported.
    She was admitted to our hospital with bleeding tendency, anemia and fever. Blood examination revealed 50 per cent promyelocytoid cells in the peripheral blood and 67.2 per cent in the bone marrow.
    Despite an intensive chemotherapy along with heparin, she died of intracranial bleeding 7 days after admission. Of the leukemic cells, 35.7 per cent were bi- and trinucleated and the rest mononuclear. Cytogenetic studies demonstrated a true tetraploid karyotype with 92 chromosomes in 60 per cent of the peripheral white cells and in 76 per cent of the bone marrow cells. The leukemic cells examined by light microscopy showed numerous fine azurophilic granules throughout the cytoplasm and transmission electron microscopy disclosed these cells to have two kinds of granules; the one with high density was smaller than mitochondria and another with low density was a little larger than mitochondria.
    Implication of the presence of tetraploidy was discussed.
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  • Tadako NAKATSUJI, Noboru MATSUMOTO, Susumu ODA, Shiro MIWA
    1980 Volume 21 Issue 2 Pages 266-272
    Published: 1980
    Released on J-STAGE: January 26, 2009
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    Three patients with acute leukemia, who have survived five years or more after the initial remission, are reported. Patient 1 (a high school girl) was diagnosed as acute lymphoblastic leukemia at the age of 15, and is still living more than 6 years. Patient 2, a 33-year-old female with acute lymphoblastic leukemia, has complete remission of five years' duration. Patient 3, a 21-year-old female with acute myelocytic leukemia, is still alive without evidence of relapse for 6 years. In these three patients, complete remission was achieved within two months by initial combination chemotherapies, and there was no relapse thereafter.
    It is noteworthy that three patients had recovered from severe infection, such as pulmonary tuberculosis (patient 1) and septicemia (patients 2 and 3) during the course of the disease. Recently, immunotherapy for patients with acute leukemia has been tried to improve remission duration and survival. In this context, it is suggested that stimulation or restoration of immunocompetence after severe infection may be one of contributing factors for prolonged remission in three patients reported herein.
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