Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
Volume 20, Issue 11
Displaying 1-15 of 15 articles from this issue
  • Michiko YAMABE, Shigeru TSUCHIYA, Tasuke KONNO, Keiya TADA, Masafumi K ...
    1979 Volume 20 Issue 11 Pages 1412-1419
    Published: 1979
    Released on J-STAGE: January 19, 2009
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    Four children, 2 boys and 2 girls aged between 3 and 8 years, with acute T-lymphoblastic leukemia (T-ALL) were reported. In all the patients thymoma and massive bone marrow infiltration of lymphoblasts were present, but anemia slight in degree. Peripheral lymphoblast counts were more than 105/mm3 in 3 cases, of which 2 had hepatosplenomegaly. A 8-year-old girl (case 3) had marked hepatosplenomegaly and subcutaneous infiltration, but 6.9×103/mm3 of peripheral white blood cells including 2% of lymphoblasts. Three of the 4 patients died within a year after the onset of the disease.
    Suface markers of the lymphoblasts were examined by the rosette formation. Features expressed by surface markers were classified with 3 groups as follows; 1. cold E (+) hot E (+) C3 (+) (case 4), 2. cold E (+) hot E (+) C3 (-) (case 3), 3. cold E (+) hot E (-) C3 (-) (cases 1 and 2). Such distinct features of lymphoblasts in T-ALL may reflect oncogenic transformation at the stage in differentiation.
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  • Fumio BESSHO, Michiko FUJIU, Hiroshi KINUMAKI, Haruko TAKEUCHI, Shunic ...
    1979 Volume 20 Issue 11 Pages 1420-1428
    Published: 1979
    Released on J-STAGE: January 19, 2009
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    Clinical pictures of hypothalmic-pituitary dysfunctuion of 33 cases of children with histjocytosis X were analysed. Some of clinical manifestations due to hypothalamic-pituitary lesions were observed in 15 children including one of 7 deceased children. Observed clinical manifestations were: diabetes insipidus in 11 cases, growth retardation in 10 cases, growth acceleration in 2 cases and precocius puberty in 2 cases. The marked female preponderance (male : female=10 : 1) in incidence of diabetes insipidus was observed. Of 11 cases with diabetes insipidus, 8 cases showed growth retardation below 2 standard deviations. The cases with diabetes insipidus showed a larger deviation from standard body height than ones without diabetes insipidus and this difference was statistically significant (P=5%). This result indicates that in histiocytosis X disease process involves rather wide region of hypothalamic-pituitary system.
    Based on an analysis of our cases, the following procedures were proposed to prevent diabetes insipidus in histiocytosis X: 1. periodical measurement of body height and ability of urine concentration should be repeated for at least 3 years after onset of histiocytosis X; 2. cases which show decreased ability of urine concentration or female children with bone lesion (s) who have onset of histiocytosis X after one year of age and show unexplained growth retardation should have radiotherapy to the hypothalamic-pituitary region.
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  • Masao TANAKA, Koichi FURUKAWA, Tsuguhiro KANEDA, Hideto MISHIMA, Yoshi ...
    1979 Volume 20 Issue 11 Pages 1429-1434
    Published: 1979
    Released on J-STAGE: January 19, 2009
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    Neoplastic cells from 16 patients with various malignant lymphomas and normal lymphoid tissue were characterized by determination of the activity of terminal deoxynucleotidyl transferase (TdT). Neoplastic cells from patients with Hodgkin lymphoma, histiocytic lymphoma were all without recognizable enzyme activity. Enzyme activity was elevated in 2 patients with lymphobalstic lymphoma (null-cel type) and in 1 out of 3 patients with diffuse, poorly differentiated lymphocytic lymphoma, with a mean activity of 13.1 units per 0.1 gram of tissue. The usefulness of TdT assay as biochemical marker of neoplastic cells in malignant lymphoma was demonstrated.
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  • Dosage Schedule of 6-mercaptopurine and Methotrexate for Maintenance of Remission
    Takeo FUJIMOTO, Keiko HASEGAWA, Yasuhiko HIYOSHI, Takashi YOKOYAMA, Py ...
    1979 Volume 20 Issue 11 Pages 1440-1450
    Published: 1979
    Released on J-STAGE: January 19, 2009
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    This controlled study of children with acute lymphocytic leukemia was designed to test the efficacy and toxicity of different mode of administration of 6-mercaptopurine and methotrexate during remission and whether more increased dosage of these drugs prolongs remission in patients with features associated with a particular poor prognosis such as initial high white cell count.
    After inducing remission with prednisolone and vincristine, patients received cranial irradiation and IT methotrexate, and were randomized to receive intermittent cyclic maintenance therapy (Regimen A, B, and C) during May 1976 and April 1977, and concomitant conbination therapy (Regimen AA, BB, and CC) during May 1977 and June 1978. Total dosage of each antileukemic drugs was designed to be equal between Regimen A and AA, B and BB, and C and CC.
    Of the 71 patients entering this study between May 1976 and June 1978, 66 (93%) attained complete remission and were randomized to receive continuation chemouherapy of Regimen A (13 cases), B (12), C (8), AA (12), BB (12), and CC (8). In Regimen A+B+C and AA+BB+CC, the results were eqivalent but 6 patients (AA: 2, BB: 2, CC: 2) died with complication of pneumocystis carinii pneumonia and varizella-zoster infection associated with severe neutropenia and mucositis during complete remission. The maintenance therapy with concomitant use of 6-MP and MTX increased toxicity and complication without demonstrably increasing the leukemocidal effect. In 16 patients with initial high white cell count over 25,000/mm3 (Regimen C and CC), the duration of complete remission and survival were inferior to those with low initial white cell count, inspite of increased dosage of 6-MP and MTX.
    These data suggested that the maintenance therapy with intermittent cyclic fashion of 6-MP and MTX might be effective and less toxic than that with concomitant conbination of these drugs, and that the additional combination chemotherapy to 6-MP and MTX might be necessary for patients with features associated with a poor prognosis such as initial high white cell count.
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  • Masako KATO, Tatsuo SHIRAI, Masanori UMEDA, Yutaka KIGA, Kazumasa MAKI ...
    1979 Volume 20 Issue 11 Pages 1451-1458
    Published: 1979
    Released on J-STAGE: January 19, 2009
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    A 56-year-old male was first examined at the Toho University Hospital because of anemia. He had a prolonged history of exposure to organic solvents until 2 years before admission. No hepatosplenomegaly was found. Blood examination revealed severe anemia, but no leukopenia or thrombopenia. Reticulocyte count was 0.1%. Bone marrow was normocellular but there was a complete depletion of all erythroid elements. A diagnosis of pure red cell aplasia was made. Basophilia and eosinophilia were observed. Serum erythropoietin level was normal. No thymoma was found. Serum vitamin B12 level was elevated. Opportunistic infections occurred in spite of normal leukocyte count. Blasts first appeared in peripheral blood samples taken on his 95th day of hospitalization, and thrombocytopenia gradually developed. He was readmitted to our hospital because of a nasal phlegmon 8 months after his first admission. At that time leukemic cells were found in his bone marrow (28.8%) and peripheral blood (9.0%). Based on the low percentage of leukemic cells, hypocellular bone marrow and cytochemical findings, we diagnosed the patient's condition to be hypoplastic acute myelomonocytic leukemia. Chromosome abnormalities appeared at this stage. After antileukemic treatment he died of pneumonia.
    Cases such as this one, (the 2nd case in Japan) showing pure red cell aplasia during the preleukemic state and progressing to acute leukemia after 8 months, are rare.
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  • Satoshi YOSHIKAWA, Hiroshi YAMAGUCHI, Kazuyuki NAITO, Tomoki NAOE, Kei ...
    1979 Volume 20 Issue 11 Pages 1459-1466
    Published: 1979
    Released on J-STAGE: January 19, 2009
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    A 53 year-old female was suffered from acute hepatitis from the end of October to the begining of December, 1977. She noticed purpura on December 31, 1977 and was admitted to Nagoya 1st Red Cross Hospital on January 4, 1978. Severe aplastic anemia was diagnosed with hematological investigations as follows: granulocyte count 396/cmm, Reticulocyte count 15,600/cmm, platelet count 16,000/cmm in peripheral blood and 27.2% hemopoietic cells in bone marrow.
    Prior to bone marrow transplantation, she was conditioned for grafting with cyclophosphamide 50mg/kg on each of four successive days. Bone marrow cells (1.13×1010) from her HLA-A, B matched and MLC negative sister (37 year-old) were infused intravenously 48 hours after the last dose of cyclophosphamide. She received intermittent methotrexate therapy after grafting to modify graft-versus-host disease (GVHD). On 12th day after bone marrow transplantation, hemopoietic cells and CFU-c in bone marrow increased in number, 80.8% and 54/2×105 cells respectively. Peripheral blood cell elements gradually increased too. Her red blood cell type (CEcS) changed to the donor type on day 68. On 85th day after marrow grafting, skin eruption (GVHD) appeard over whole body and lasted for about one and a half month.
    She is alive in good hematological condition over one year after bone marrow transplantation.
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  • Katsuyo YAMAUCHI, Masamitsu INOUE, Susumu ODA, Noboru MATSUMOTO, Shiro ...
    1979 Volume 20 Issue 11 Pages 1467-1474
    Published: 1979
    Released on J-STAGE: January 19, 2009
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    Two fatal cases of aplastic anemia which developed after administration of gold salt for the treatment of rheumatoid arthritis were reported. The frst case was a 58-year-old female who had received aurothioglucose 390 mg totally during the period of 5 months. On admission, serere pancytopenia (platelet 7000/mm3, leucocyte count 900/mm3 with 1% nuetrophil) was noted. No recovery was obtained in spite of anabolic steroid therapy and she expired on the 15th hospital day from septicemia and intracranial bleeding. The second case was a 43-year-old housewife who had received sodium aurothiomalate 600mg totally during the period of 8 months. And then bleeding tendency developed. She had marked pancytopenia (Hb 5.0g/dl, leucocyte count 1200/mm3 with 6% neutrophil, platelet 8000/mm3) and severe hypoplastic bone marrow. Any treatment, including BAL, lithium, and anabolic steroid, failed to improve bone marrow findings. She died four months later due to septicemia and subarachnoidal hemorrhage.
    As far as we reviewed the recent literatures, 14 cases of aplastic anemia induced by gold salt therapy have been reported. The mortality rate was high (64.3%).
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  • Hiroshi MORI
    1979 Volume 20 Issue 11 Pages 1475-1481
    Published: 1979
    Released on J-STAGE: January 19, 2009
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    A 47 year-old male was admitted to hospital on April 11 with general fatigue, anorexia and ecchymosis of lower extremities. He was well unti 3 months ago when he complained of recurrent upper respiratory infection and diarrhea. In February 1978, proteinuria and renal dysfunction were first realized.
    On examination he looked pale and ecchymosis and pitting edema were proved. His blood pressure was 236/126, with retinopathy (stage IIb∼IIIa Keith-Wagener). The Hb was 6.1g/dl with reticulocytosis 44‰, white cell count was 6,140, platelet count was 25,000, and red cell fragmentation remarkably appeared on peripheral blood smear. His bone marrow aspirate revealed erythroid hyperplasia with normal megakaryocyte counts. The BUN 93mg/dl, serum creatinine 9 mg/dl, LDH 2,160U. Coagulation parameters were normal except the presence of FDP. The urinalysis revealed protein, red cell and platelets.
    After starting combined treatment of aspirin 1g P. O. and dipyridamole 200mg i. v. d., with peritoneal dialysis, the platelet count increased to 100,000 within a few days. But the platelet count soon decreased to 23,000 after discontinuity of these platelet inhibitors because of gastrointestinal bleeding. Then dipyridamole was given 100mg i. v. d. again, platelet count rapidly increased to 100,000, and red cell fragmentation completely disappeared.
    It was concluded that inhibitors of platelet function with dialysis revealed definite benefit for an adult case of hemolytic uremic syndrome.
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  • Manabu MUSASHI, Kyoichi TOTSUKA, Asako WAKATA, Mariko MINETA, Hirotaka ...
    1979 Volume 20 Issue 11 Pages 1482-1491
    Published: 1979
    Released on J-STAGE: January 19, 2009
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    An autopsied case of nodular sclerosis (Hodgkin's disease with terminal leukemic change) is reported. A 53-year-old man had a high fever 3 months before admission. After 2 cervical lymph node biopsies, the patient was diagnosed as having nodular sclerosis (Hodgkin's disease). Based on the results of lymphangiography, a 67Ga-tumor scan, a liver biopsy, a bone marrow biopsy, and other clinical examinations on July 12, 1977, he was determined as being at stage Ib. Cervical and supraclavicular lesions were treated with radiation (4,000 rads), and the results were positive. Five months after diagnosis, he suffered from fever, hepatosplenomegaly, and pancytopenia. He was admitted again on May 15, 1978. Some Hodgkin's cells were found in a bone marrow smear, and he was detemined as being at stage IVb. OPP therapy was given, but the response was negative. Two days before his death, Hodgkin's cells (7%) were found in a routine peripheral blood smear. He died on March 29, 1978. Autopsy revealed lymphadenopathy and hepatosplenomegaly, with marked infiltration and proliferation of Hodgkin's cells and Reed-Sternberg's cells. These changes were also seen in the bone marrow, pancreas, lungs, etc. Hodgkin's disease with leukemic change is summarized, and whether nodular sclerosis, which had been thought to have a good prognosis, may develop to leukemic change or not, are discussed.
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  • H. KOBASHI, I. MIYOSHI, I. KIMURA, J. SAKATO, S. SAINO, H. SUWAKI, S. ...
    1979 Volume 20 Issue 11 Pages 1492-1496
    Published: 1979
    Released on J-STAGE: January 19, 2009
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    Hypoplastic anemia in a 39-year-old woman probably caused by chlorpromazine is reported. The patient visited a hospital because of fever and petechiae in July, 1976. For 13 years, she had been treated with chlorpromazine, trifluoperazine and levomepromazine for schizophrenia. The initial blood study revealed Hb 4.6g/dl, RBC 160×104/mm3, Ht 13%, WBC 1,200/mm3 and platelet count 1.7×104/mm3. The sternal bone marrow was hypoplastic with 74.2% lymphocytes. The patient was given transfusion of blood and platelets and treated with prednisolone and oxymetholone. About 3 months later, the hematologic finding gradually improved and a remission was obtained in May, 1977.
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  • Jun-ichi AKATSUKA, Takuo HIROTSU, Eiji TOMITA, Misao TAMARU, Takahiro ...
    1979 Volume 20 Issue 11 Pages 1497-1504
    Published: 1979
    Released on J-STAGE: January 19, 2009
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    A 9 years 2 months old male without consanguinity, was Shown to have congenital factor XIII deficiency whose initial bleeding episodes whre umblical bleeding and cephalohematoma in his newborn period. Thereafter, repeated abnormal bleedings were observed at every traumatic accident and stopped with fresh blood transfusions. At the first visit to our clinic, physical examination revealed tumor of right upper arm due to fresh intramuscular bleeding and ugly scar formation at the forehead and the right femoral area whcih were produced after the surgical manipulation given for hemostatic control at the past bleeding episodes.
    Hematologic and coagulation studies revealed no anemia and normal liver function. All of bleeding time, platelet count, PT, PTT and tournique test fell within the normal range. However, remarkable reduction of ma and mε of his thromboelastogram and the increased solubility of patient's clot in 5M urea (75 min.) and 1% monochloracetic acid (within 15 min.) were found. The assay of factor XIII of the patient's plasma was below 1% of normal level by the neutralization test using antiserum of factor XIII and assay of subunits a and b in his plasma were 0% and 45%, respectively Both factor XIII levels of his parents were 50% of normal.
    Excellent hemostatic control was obtained by infusion of factor XIII concentrate (500 units) and its half life was three days. The thromboelastogram was also kept within normal range over two weeks after infusion of the concentrates.
    Subunit b/a ratio was ∞ in homozygous patient, 1.49 in heterozygous (carriers) and 1.07±0.13 (m±SD, n=9) in control. Analysis of subunit ratio may provide useful method to detect the carriers.
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  • Kichiro OSAMURA, Shunyo KANAYAMA, Hideaki YAMAZAKI, Tetsuo NAKATA, Aki ...
    1979 Volume 20 Issue 11 Pages 1505-1511
    Published: 1979
    Released on J-STAGE: January 19, 2009
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    A 55-year-old male was admitted to our hospital with lymphadenopathy and high fever in January, 1978. Lymphocytic predominant type of Hodgkin's disease was revealed by the biopsy. He was treated with 4 times of combination chemotherapy, VEMP, and next 4 times treated with VENP. OK-432 has been added on the therapy since March. After these therapy, lymphadenopathy disappered except for 2 lymph nodes of left neck. As localy treatment, 5 or 10 KE of OK-432 were injected into these two lymph nodes. Two weeks later, by the biopsy, amyloidosis was revealed with a few of Hodgkin cells in these lymph nodes treated with OK-432. However, histologicaly same findings were observed in axillar and inguinal lymph nodes. Moreover, amyloid deposit was revealed in rectal muscle, but not in liver tissue. After the treatment of more 4 times of VENP, all lymphadenopathy disappeared.
    Several causes are suspected to explain amyloidosis in this case as follows:
    1) The same immunological abnormality which is suspected to induce Hodgkin's disease.
    2) Reduced immune response with Hodgkin's disease and/or following combination chemotherapy.
    3) Side effect of OK-432.
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  • Kiyoshi IRIMADA, Shinkiti YOKOYAMA, Yoozo YOSHIMURA, Tetsu SAITOH, Tad ...
    1979 Volume 20 Issue 11 Pages 1512-1519
    Published: 1979
    Released on J-STAGE: January 19, 2009
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    A male patient, aged 40 day, was admitted to our hospital in October, 1977, for evaluation of skin nodules noticed at birth. On admission, a hematological examination showed no leukemic change. Skin biopsies were performed, but no diagnosis was established. At the age of two months, leukemic cells appeared in the peripheral blood and bone marrow. Leukemic cells showed varying degrees of activity for peroxidase, non-specific esterase, and intracytoplasmic muramidase, and showed latex-particle phagocytosis in vitro and erythrophagocytosis in the tissue section. From examination of the cytochemical patterns as well as from the morphological features a diagnosis of acute monocytic leukemia was made.
    Shortly after initiation of remission induction therapy, the skin nodules diminished in size, but the patient died of pneumonia and sepsis.
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  • Shigeyoshi MATSUDA, Moritoshi KYUTOKU, Chieko NOGUCHI, Makoto SAKAMOTO ...
    1979 Volume 20 Issue 11 Pages 1520-1525
    Published: 1979
    Released on J-STAGE: January 19, 2009
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    Intracellular inclusion bodies resembling Auer rod in palsma cells from a patient with rectal cancer were cytochemically and electronmicroscopically studied.
    Cytochemical findings revealed that these inclusion bodies were strongly positive both for acid phosphatase and β-glucuronidase staining, but were negative for the staining with peroxidase, Sudan black B, PAS and nonspecific esterase. They had no activity either by Congo red and Thioflavin-T staining, indicating that they were not amyloid.
    In electronmicroscopic observation, the inclusion bodies were surrounded by single limiting membrane in rough-surfaced endoplasmic reticulum or in Golgi apparatus. These Auer rod like inclusions were found to be hexagonal crystalloid in the cross section, and showed 50 Å periodicity between light and dense lines in the longitudinal section.
    These results suggest that the observed inclusion bodies are lysosome.
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