臨床血液
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
24 巻, 8 号
選択された号の論文の19件中1~19を表示しています
第24回総会
招待講演
臨床研究
  • —自験9症例を中心に—
    松村 隆文, 橋田 哲夫, 江角 典子, 中川 雅生, 木崎 善郎, 日比 成美, 小椋 香苗, 今宿 晋作, 佐藤 典子, 小西 清三郎, ...
    1983 年 24 巻 8 号 p. 996-1002
    発行日: 1983年
    公開日: 2009/01/26
    ジャーナル 認証あり
    Nine cases of histiocytic medullary reticulosis in childhood were studied. Ages ranged from 10 months to 13 years-5 months with median 2 years-11 months and four cases were male. Bone marrow smears, obtained on admission and periodically during the treatment, were stained with Wright-Giemsa. Marrow histio-monocytes were classified according to the definition of Manoharan, depending on the degree of maturation and phagocytic activity. Histio-monocytes were graded from I through V, and pre-treatment distribution patterns were compaired for the nine cases, which resulted in two distinct groups: A (n=5) and B (n=4), respectively. Group A demonstrated higher percentage of immature I and II type cells (32.6±3.8%); by contrast, group B was characterized by lower I and II type cells (13.3±4.2%). Follow-up of marrow histio-monocytes studied in some cases during the course of treatment reflected the tumor activity. In spite of the fact that the two groups could not be clearly categorized by clinical symptoms and signs, and routine laboratory procedures, the four cases in the group A responded poory to the induction chemotherapy, with one CR, two PR, one NR and one died before treatment. On the other side, all four cases in the group B achieved CR. In these nine cases, determination of the pre-treatment distribution patterns of marrow histio-monocytes was significantly useful in predicting the prognosis of histiocytic medullary reticulosis in childhood.
  • 林 泰秀, 阿部 緑生, 三比 和美, 桜井 雅温, 服部 理男, 森 泰二郎, 中澤 眞平, 水谷 修紀, 別所 文雄
    1983 年 24 巻 8 号 p. 1003-1011
    発行日: 1983年
    公開日: 2009/01/26
    ジャーナル 認証あり
    We examined chromosomes, surface immunoglobulins (SIg) and EBV-determined nuclear antigen (EBNA) in five Japanese patients with Burkitt's lymphoma (BL). Three patients had a translocation, t (8; 14) (q24; q32). One other patient had a 14q+ chromosome but the origin of the translocated segment in this patient could not be determined. The other patient had a variant translocation, t (2; 8) (p11; q24). These abnormalities are the same as those which have been reported in endmic as well as in nonendemic BL. Four patients had additional abnormalities, including a partial duplication of the long arm of a No. 1 or a partial deletion of the long arm of a No. 6 chromosome (6q-). The latter finding confirms the earlier observation that not only patients with T cell or null cell but also ones with B cell lymphoma-leukemia can have a 6q- chromosome. Three patients with the t (8; 14) were all children. Their tumor cells had SIgM but did not harbor EBNA. The patient with the unidentified 14q+ was diagnosed having B cell ALL-L 3. In the patient with t (2; 8), no SIg light chain was detected on the surface of the tumor cells, while EBNA was present in them. The variant translocation t (2; 8) suggests that the break at 8q24 is more important than that at 14q32 in the genesis of BL. Possible difference in the histology, clinical features and prognosis between BL's with t (8; 14) and those with a variant translocation remains to be elucidated.
  • —臨床的及び実験的研究—
    杉原 尚
    1983 年 24 巻 8 号 p. 1012-1019
    発行日: 1983年
    公開日: 2009/01/26
    ジャーナル 認証あり
    Hemolytic effect of glycerol was investigated in vivo and in vitro in human red cells. After administration of glycerol in vivo, the increase of plasma free hemoglobin concentration was observed concomitant to the decrease of red cell membrane lipids. The lipids at the outer surface (free cholesterol, phosphatidyl choline and sphingomyelin) were decreased markedly more than those at the inner surface (phosphatidyl ethanolamine and phosphatidyl serine), dependent on the concentrations of glycerol used. The findings observed in vivo were verified by the in vitro experiments. To prevent the glycerol induced hemolysis, the compound should be infused at the rate of less than 3.3 ml/hour/kg with the interval longer than 6 hours in each infusion.
    On the other hand, the abnormal Heinz body formation with acetylphenyl hydrazine (the Beutler's method) was observed in these red cells incubated with glycerol. Glycerol itself did not affect the hemoglobin metabolism and also the activities of glycolytic enzymes. It is most likely that glycerol induces the red cell membrane lipid abnormalities at the outer leaflets which will cause the increased permeability of acetylphenyl hydrazine, resulting the increase of Heinz body formation in the normal red cells incubated with glycerol.
  • 竹村 譲, 外山 圭助, 東 敏昭
    1983 年 24 巻 8 号 p. 1020-1025
    発行日: 1983年
    公開日: 2009/01/26
    ジャーナル 認証あり
    Six adult long-term (more than 6 years) survivors with acute leukemia treated at the Keio University Hospital since 1970 were analyzed in detail in comparison with patients of acute leukemia who died within 5 years.
    Four of the six patients had M 2 of the FAB classification (differentiated myeloblastic type), 3 of whom were over 49 year-old. The remaining two were young, and had L 1. Prior to the initiation of therapy, serum lactate dehydrogenase (LDH) levels were significantly (p=0.05) lower in the long-term survivors than other patients. Induction chemotherapies in the six patients were variable, but complete remission were easily achieved in each case. One patient of L1 receiving insufficient maintenance therapies repeated relapses of leukemia and ultimately died. Another patient of L1 developed a CNS relapse 98 months after diagnosis, but reattained complete remission. In two of these six patients aggravation of old tuberculosis was seen in the course of treatment to leukemia. It might suggest that enhanced immunological mechanisms of hosts by tuberculosis took part in long-term survival of acute leukemia.
  • 松尾 武文, 大木 康雄, 小林 弘子, 山田 勤
    1983 年 24 巻 8 号 p. 1026-1031
    発行日: 1983年
    公開日: 2009/01/26
    ジャーナル 認証あり
    Characterization of congenital antithrombin III (AT III) deficiency was determined with 2 dimensional crossed immunoelectrophoresis (CIE). CIE of a family member showed that the fast moving peak composed of AT III-heparin complex became smaller than normal plasma when CIE ran with high molecular heparin fraction.
    However, the fast moving peak became bigger when that ran with low molecular heparin fraction. Especially, the family member (propositus' brother) was found to have considerably the large fast moving peak. It was thought as one of the reasons why his AT III showed normal heparin cofactor activity in spite of a half of it's antigen level.
    CIE was determined after the infusion of AT III concentrate in propositus. As the increased level of it's antigen, the slow moving peak in CIE became bigger than original CIE. Also, it was suggested that there was abnormal AT III with low affinity to heparin in plasma's AT III after the infusion of concentrate. But the discrepancy between the functional activity and antigen level could not be found in the plasmas after AT III concentrate infusion.
    Transient abnormality in CIE after the concentrate was shown to have the same electrophoretic heterogeneity as appearing under Sas' classification of 'classical' congenital AT III heterogeneity.
  • —関東地区のデータより—
    飛内 賢正, 永井 雅巳, 湊 啓輔, 下山 正徳
    1983 年 24 巻 8 号 p. 1032-1038
    発行日: 1983年
    公開日: 2009/01/26
    ジャーナル 認証あり
    Serum or plasma specimens from 340 patients with lymphoid malignancies and benign diseases were screened for reactivity with adult T-cell leukemia virus-associated antigen (ATLA) by indirect immunofluorescence assay. Anti-ATLA antibodies were found in 24 (7.1%) of the total 340 patients, but in 13 (41.9%) of 31 patients born in the ATL-endemic area. The antibodies were detected in 15 (88.2%) of 17 patients with ATL or adult T-cell leukemia/lymphoma, and nine of them were born in the ATL-endemic area. Among T-cell malignancies, the antibodies were detected in none of T-lymphoblastic lymphoma, IBL-like T-cell lymphoma, T-CLL and mycosis fungoides, and birthplaces of them were distributed concentrically around Tokyo. In contrast, birthplaces of the patients with ATL and anti-ATLA positive T-cell lymphoma were clustered in Kyushu, the Pacific coast of Kanto and Tokai, and Iwate prefecture. In ATL-nonendemic area, anti-ATLA is considered to be a very useful marker for identifying ATL and ATLV-related T-cell lymphoma.
  • 丹羽 靱負, 尾崎 元昭
    1983 年 24 巻 8 号 p. 1039-1048
    発行日: 1983年
    公開日: 2009/01/26
    ジャーナル 認証あり
    The capacity to generate oxygen intermediates (OI; O2-, H2O2, OH·) and chemiluminescence, and to release lysosomal enzyme (lysozyme, β-glucuronidase), and the superoxide dismutase (SOD) activity of polymorphonuclear leukocytes (PMNL) and monocytes from 14 leprotic patients manifesting a bacillary index above 2.2 was examined to determine the action mechanism of clofazimine. Significantly enhanced SOD activity, and a decrease in O2-, and OH· production were observed in the patients with more than 4 years history. The generation of OH· was significantly increased, in a dose dependens manner, by clofazimine, with a subsequent decrease in H2O2 and chemiluminescence, while SOD activity of the PMNLs and monocytes was not affected. In the medium supplemented with FeSO4 or EDTA containing Fe++, OH· production was further markedly elevated by the drug. Phagocytic SOD in PMNLs and monocytes of the pasients was both host- and bacillus-derived, because the presence of potassium cyanide, to which human-derived cuprozic SOD is susceptible, did not completely abrogate SOD activity. The difficulty in treating leprosy may be partly ascribable to the decreased phagocytic OH· generation in this disease, which in leprosy patients is induced by increased Hansen bacillus-derived SOD uptaken by the patients. Clofazimine may be effective in leprosy by potentiating the catalyzing activity of Fe++ which facilitates Haber-Weiss reaction to increase OH· formation from H2O2, without affecting SOD activity which was enhanced by their uptake.
  • 前田 秀明, 内海 真, 堀田 知光, 山田 英雄
    1983 年 24 巻 8 号 p. 1049-1054
    発行日: 1983年
    公開日: 2009/01/26
    ジャーナル 認証あり
    We have investigated the in vitro effects of fluoxymesterone on erythroid colony growth from bone marrow of eight normal subjects and sixteen patients with aplastic anemia. Seven patients were androgen dependent, three were androgen effective, and six were androgen ineffective by previous treatment. CFU-E assay was carried out using the plasma clot method with 2×105 marrow cells/dish, 1.0 U/ml of erythropoietin, and various concentrations of fluoxymesterone.
    It was demonstrated that the number of erythroid colonies from marrow of androgen dependent patients was significantly increased with the addition of fluoxymesterone in comparison with that of normal subjects. On the other hand, the number of erythroid colonies from marrow of androgen ineffective patients was not significantly increased compared to that of normal subjects.
    The results may suggest that there exist unique CFU-E clones which are very sensitive to androgens in vitro as well as in vivo in the patients with androgen dependent aplastic anemia. In addition, it has been revealed that in vitro fluoxymesterone sensitivity test may be a valuable indicator to predict the response to androgen therapy in the patients with aplastic anemia.
症例
  • —その赤血球変形能について—
    荒木 純子, 巽 陽一, 三宮 祐一, 吉川 岳男, 橋本 孝二, 瓦林 佳子, 朴 勤植, 紙谷 富夫, 任 太性, 巽 典之, 奥田 清 ...
    1983 年 24 巻 8 号 p. 1055-1059
    発行日: 1983年
    公開日: 2009/01/26
    ジャーナル 認証あり
    Recently we found a case with chorea-acanthocytosis characterized by decreased deformability of red blood cells. The deformability of red blood cells was determined by the capillary tube centrifugal technique and rheocytometry. The former technique revealed that a normal discoid red cell resembled a handbag like form, while an acanthocyte took the mitten-like form. Rheocytometry disclosed that the index of deformability was significantly lower in the patient than in normal individuals. The decrease in deformability may be caused by a fall in viscoelasticity of the erythrocyte membrane. The present observation would present an important key to clarify the nature of this disease.
  • 大地 信彰, 稲永 隆, 前田 利朗, 鈴木 久三, 日野 由和夫, 斉藤 英彦, 吉富 誠子
    1983 年 24 巻 8 号 p. 1060-1067
    発行日: 1983年
    公開日: 2009/01/26
    ジャーナル 認証あり
    A 43-year-old woman was admitted because of her severe hypertension and cerebral thrombosis.
    Past history was remarkable in that she had repeated episodes of ulcer formation in her toes for last two years.
    Physical examination on admission showed an ulcer in a left toe.
    Laboratory data including CBC, NAP score and chromosome analysis were consistent with the diagnosis of primary thrombocythemia.
    Aortogram demonstrated severe stenosis at the beggining of a left renal artery.
    Marked elevation of plasma renin activity was found in the left renal vein.
    The administration of captopril (an angiotensin I converting-enzyme inhibitor) from the 5th hospital day resulted in prompt normalization of blood pressure and complete healing of the refractory toe ulcer was achieved after a few weeks.
    Captopril as a potent vasodilator appeared to be efficacious for the treatment of the refractory ulcer in this patient.
  • 恵美 宜彦, 渡辺 英二, 加藤 幸男, 川島 康平, 神谷 忠, 大野 竜三
    1983 年 24 巻 8 号 p. 1068-1072
    発行日: 1983年
    公開日: 2009/01/26
    ジャーナル 認証あり
    A long survivor of ALL who showed overt CNS-leukemia and 8 hematological relapses durng 12 years is reported. A 9-year-old boy was admitted to our hospital in October, 1970 with the presenting symptoms of easy fatigability and paleness of the face. A diagnosis of ALL was made and the patient was placed on a combination chemotherapy of DVP, which brought a complete remission in January, 1971. Twenty-three months later the patient developed overt CNS-leukemia. Being treated with frequent intrathecal chemotherapy and irradiation, the patient had leukoencephalopathy as permanent sequela. From that time on, the patient repeated 8 hematological relapses in October, 1973, September, 1976, May, 1978, November, 1979, October, 1980, February, 1981, December, 1981 and July, 1982. After 12 years under therapy the patient died of sepsis in October, 1982.
  • 赤星 雅, 長原 光, 片平 潤一, 押味 和夫, 溝口 秀昭, 岡田 美智子, 榎本 康弘, 渡辺 陽之輔, 中沢 眞平
    1983 年 24 巻 8 号 p. 1073-1079
    発行日: 1983年
    公開日: 2009/01/26
    ジャーナル 認証あり
    A case of CML terminating in megakaryoblastic crisis is reported.
    A 30-year-old female was admitted to our hospital because of high fever. Three months before admission, she was diagnosed as having CML from the findings of leukocytosis in the peripheral blood, low NAP score and presence of Ph1 chromosome, and treated with busulfan thereafter. Physical examination on admission revealed marked hepatosplenomegaly, lymphadenopathy and skin eruptions.
    The white cell count in the peripheral blood was 100,000/μl with 81% blast cells. The blast cells exhibited a uniform size of 20 μm in diameter and had a large nucleus with homogeneous chromatin, and 1 or 2 nucleoli. The cytoplasm was abundant, basophilic and contained few granules. A few cells showed cytoplasmic blebs. The blast cells were peroxidase-negative, PAS-negative, α-naphthyl acetate esterase-positive, and acid phosphatase-positive. Ultrastructural studies revealed that the blast cells contained platelet-peroxidase in the endoplasmic reticulum and nuclear membrane distinct from myeloperoxidase. From these findings, she was diagnosed as having CML terminating in megakaryoblastic crisis.
    She did not respond to a combination chemotherapy including vindesine and prednisolone, and died one month and a half after admission.
    It is interesting to note that chromosomal abnormalities such as duble Ph1, 8 trisomy and t(1; 3) were observed. The relationship between megakaryoblastic crisis and t(1; 3) was discussed.
  • 恵美 宜彦, 川島 康平, 大野 竜三, 平林 紀男, 矢野 邦夫, 竹山 英夫, 坂 行雄, 佐竹 立成
    1983 年 24 巻 8 号 p. 1080-1085
    発行日: 1983年
    公開日: 2009/01/26
    ジャーナル 認証あり
    A 51-year-old male was admitted to Nagoya Ekisaikai Hospital with complaints of a high fever, cough, and left chest pain. Physical examination revealed moderate epigastric tenderness, decreased vocal fremitus and flat percussion note on the left side with decreased breath sounds. The liver edge was palpable 2 finger-breadths below the right costal margin. No splenomegaly nor lymphadenopathy was noted. X-ray films of the chest showed left pleural effusion. Immunoelectrophoresis revealed a monoclonal gammopathy in the serum with 8,560 mg/dl of IgM-λ and Bence Jones protein in the urine. The bone marrow picture disclosed 14.5% plasma cells, and osteolytic lesions were found in the cranial bone. Gastric biopsy revealed massive plasma cell infiltration in the lamina propria. By means of immunohistochemical staining, the cytoplasms of the plasma cells were stained by anti-μ and anti-λ antibodies. These results indicated the infiltrating cells were monoclonal.
    From these findings the patient was diagnosed as primary macroglobulinemia occurred in the stomach and treated by interferon, antineoplastic agents and plasmapheresis which entirely improved clinical complaints.
  • 安井 耕三, 山崎 宗廣, 宮川 幸昭, 小宮山 淳, 赤羽 太郎
    1983 年 24 巻 8 号 p. 1086-1091
    発行日: 1983年
    公開日: 2009/01/26
    ジャーナル 認証あり
    We reported three children with lazy-leukocyte syndrome who had suffered from recurrent pyogenic infections and stomatitis since early infancy.
    They were unrelated each other and their family history was unremarkable.
    All of the patients had severe neutropenia (59∼204/cmm) despite normal peripheral leukocyte counts, and their neutrophils were defective in chemotaxis and random mobility.
    The bone marrow pictures were normal: there were normal numbers of neutrophils at every stage of maturation and their morphology was normal.
    Humoral and cellular immunity tests such as serum IgG, A and M levels, T and B subpopulations, lymphocyte blastogenesis, and PHA skin test were normal.
    Lymphocyte derived chemotactic factor (LDCF) production studied in one of them was normal.
    Further hematologic and immunologic studies are needed to disclose the mechanism of the defective neutrophil mobility in this syndrome.
  • 米満 博, 近藤 春樹, 奥田 邦雄, 遠藤 伸行, 山口 覚太郎
    1983 年 24 巻 8 号 p. 1092-1097
    発行日: 1983年
    公開日: 2009/01/26
    ジャーナル 認証あり
    A 67-year-old woman was diagnosed as having polycythemia vera in 1966, and was treated with phlebotomy alone for half a year after diagnosis. The patient remained asymptomatic without any treatment during the following 14 years. Leukoerythroblastic anemia and enlargement of the spleen developed in 1981, and she was put on anabolic steroids, and blood transfusion therapy was started. She was readmitted on July 19, 1982, because of shortness of breath. On admission, she had massive hepatosplenomegaly and leukoerythroblastic anemia with tear drop poikilocytosis. The neutrophil alkaline phosphatase score was within normal limits. The chromosome analysis of peripheral blood revealed a normal karyotype. Bone marrow biopsy from the iliac crest showed osteomyelosclerosis. A diagnosis of polycythemia vera terminating in myelofibrosis was made.
    In the current case, myelofibrosis should have developed as a terminal event during the evolutional course representing a natural history of this disease.
  • 岩井 艶子, 二宮 恒夫
    1983 年 24 巻 8 号 p. 1098-1105
    発行日: 1983年
    公開日: 2009/01/26
    ジャーナル 認証あり
    A 1-year-2-month old boy was admitted to the Tokushima University Hospital because of petechiae and nasal bleeding. The peripheral blood showed a decrease in platelets and the bone marrow showed an increase in immature megakaryocytes. He was diagnosed as idiopathic thrombocytopenic purpura (ITP). Therapy with prednisolone for 2 months resulted no hematological improvement. After discharge he attended the outpatient clinic about once a month for hematological examinations, but thrombocytopenia persisted.
    At the age of 4 years he complained fatigability. Physical examination revealed no remarkable abnormalities. The peripheral blood revealed an increase in mature eosinophils and platelets. The bone marrow was entirely normal except for an increase of mature eosinophils. Repeated examination of stools did not reveal any ova and parasites. The sedimentation rate was 51 mm/hr and rheumatoid factor was positive. Serum immunoelectrophoresis disclosed a marked increase of IgE-gloubulin. A chest X-ray film showed infiltrates at right lung fields. The number of CFU-EO in the patient was greater than that of the healthy subjects, and the production of EO-CSF produced by mononuclear cells was not increased.
    Prednisolone therapy resulted in an disappearance of his complaints, and the abnormal results in laboratory examinations gradualy improved by the age 6 years.
    This patient with hypereosinophilic syndrome are considered to be unique because of simultaneous increase of platelets in the clinical course of chronic ITP.
  • 安田 佳織, 名取 英世, 山口 達夫, 田中 健, 江上 康一郎, 加地 正郎, 赤木 弥寿子, 森松 稔
    1983 年 24 巻 8 号 p. 1106-1112
    発行日: 1983年
    公開日: 2009/01/26
    ジャーナル 認証あり
    A case of sepsis accompanied with foam cell syndrome in bone marrow was reported. A 66-year-old woman admitted to our hospital because of fever and general fatigue on November 25, 1981. Cultures of blood and urine were positive for E. coli, so she was diagnosed as sepsis and treated with antibiotics. After two weeks anemia and leukopenia rapidly progressed. A bone marrow aspirate was hypocellular with increased number (19.2%) of foam cells with erythrophagocytosis and scattered leukophagocytosis. Cells similar to those characterised as the “sea-blue histiocyte” were not observed. An ultrasonography showed slightly enlarged spleen. She became progressively debilitated and died on December 31. On autopsy, there was no evidence of the previously noted foam cells in bone marrow, spleen and other organs.
    The relationship of this foam cell syndrome associated with E. coli infection to prominent hemophagocytosis was discussed.
例会
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