巻号一覧
26 巻, 5 号
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第26回総会
シンポジウムI エリスロポエチンの基礎と臨床
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浦部 晶夫, 千葉 省三, 高久 史麿1985 年 26 巻 5 号 p. 640-645
発行日: 1985年
公開日: 2009/02/02
ジャーナル 認証ありAssay methods for erythropoietin (EPO) include an in vivo bioassay, an in vitro bioassay, a hemagglutination inhibition assay, and a radioimmunoassay. The in vivo bioassay is the most fundamental and reliable method, but it is less sensitive and is a tedious and time-consuming procedure. The in vitro bioassay using fetal mouse liver cells is more sensitive and reliable as well. However, bioassays may detect the sum of several erythropoietic stimulatory activivies and some inhibitory activities. Thus the development of a radioimmunoassay system has been long awaited. In this paper, outlines of each method for determination of EPO including a radioimmunoassay were described. Serum EPO titers of various hematological conditions by a radioimmunoassay and by an in vitro bioassay were also described.抄録全体を表示PDF形式でダウンロード (397K) -
佐々木 隆造1985 年 26 巻 5 号 p. 646-650
発行日: 1985年
公開日: 2009/02/02
ジャーナル 認証ありA stable hybridoma clone that secretes monoclonal antibody against human erythropoietin (EPO) was established. EPO was isolated from urine of aplastic anemic patients in a high yield with a simple purification procedure using an immunoadsorbent column of the monoclonal antibody and a Sephadex G-100 column. About 6 mg of EPO was isolated from 600 liters of urine and the specific activity was estimated to be 81,600 units/mg of protein with an in vivo radioiron incorporation assay method, using starved rats. Electrophoretic microheterogeneity of purified EPO is due to variable amounts of sialic acid in carbohydrate side chains attached to EPO molecules.抄録全体を表示PDF形式でダウンロード (377K) -
高橋 正知, 溝口 秀昭1985 年 26 巻 5 号 p. 651-660
発行日: 1985年
公開日: 2009/02/02
ジャーナル 認証ありWe investigated the effect of the purified erythropoietin (Ep) (specific activity 81,000 U/mg protein) on hemopoietic progenitor cells such as CFU-C, CFU-E, BFU-E and CFU-M.
As a result, the purified Ep has no colony-stimulating activity for human and murine CFU-C.
The purified Ep stimulated the erythroid colony formation derived from both human and murine CFU-E. The purified Ep also stimulated the colony formation derived from both human and murine BFU-E. When the concentration of fetal calf serum in the medium was decreased to 10% in order to decrease the concentration of BPA, very few bursts were noted by the addition of purified Ep. Moreover, the addition of the boiled purified Ep with the untreated purified Ep did not stimulate burst formation, although the addition of boiled crude Ep with purified Ep stimulated burst formation. These results suggest that the purified Ep has little BPA, although the crude Ep has BPA.
The purified Ep stimulated murine megakaryocyte colony formation in the plasma clot. However, the purified Ep did not stimulate murine megakaryocyte colony formation in serum free conditions. These results suggest that the purified Ep dose not have megakaryocyte colony-stimulating activity, but has the activity of megakaryocyte potentiators.
Finally the injection of the purified Ep did not increase the total number of CFU-C and CFU-M, but increased that of CFU-E and BFU-E.抄録全体を表示PDF形式でダウンロード (768K) -
寺澤 崇1985 年 26 巻 5 号 p. 661-667
発行日: 1985年
公開日: 2009/02/02
ジャーナル 認証ありClonal cell culture method has made it possible to analyze the expression of certain types of proteins during the course of hemopoietic cell diffrentiation and development as well as to detect the kinetics of hemopoietic precursors at various hematological conditions. In this section, we would like to introduce the influence of erythropoietin (Epo) and burst promoting activity (BPA) on fetal hemoglobin (Hb F) biosynthesis by adult erythropoietic precursors (BFU-E) in culture and the special charateristics of erythroid precusors involved in Gγ and Aγ globin chains synthesis observed in juvenile chronic myelogenous leukemia (J-CML).
In case of sheep plasma Epo (14 U/mg) and human urinary Epo (40 U/mg), the correlation between the levels of Epo and the relative rates of Hb F synthesis by BFU-E was observed. However, human pure Epo (70,400 U/mg) showed no Epo-dose dependence against Hb F augmentation. BPA both from bone marrow and T-cell conditioned media significantly stimulate Hb F synthesis only at low fetal calf serum concentrations (10∼15%). These results may suggest that BPA rather than Epo is an important regulator for Hb F augmentation by adult erythropoietic precursors in culture.
On the basis of concept of cellular regulation of Hb F synthesis in vitro, we try to investigate the pathogenesis of fetal like hemopoisis manifested in J-CML by the analysis of the biosynthetic ratio of Gγ and Aγ globin chains in the erythropoietic bursts. The Gγ and Aγ ratio of Hb F synthesized in the erythropoietic bursts revealed a high Gγ synthetic pattern similar to that observed in fetal liver BFU-E. Therefore, the findings suggest that fetal like erythropoiesis in J-CML is regulated by the erythropoietic precursors, in which normal hemoglobin swiching processes has not occured.抄録全体を表示PDF形式でダウンロード (557K) -
漆山 勝, 森山 美昭1985 年 26 巻 5 号 p. 668-673
発行日: 1985年
公開日: 2009/02/02
ジャーナル 認証ありIn order to clarify the mechanisms of anemia of chronic renal failure (CRF) and also to evaluate the clinical use of erythropoietin (Epo) to patients undergoing hemodialysis, we investigated erythropoiesis in uremia and the response to Epo of erythroid progenitor cells in the presence of uremic sera in vitro.
Most of sixty-three dialyzed patients studied in our study remained anemic (mean hematocrit level: less than 30%). Anemia of these patients was associated with “ineffective erythropoiesis” because of a good correlation between anemia and reduced reticulocyte count (p<0.01, r=0.5001), and of bone marrow erythroid hyperplasia in uremia. Serum Epo level measured by FMLC assay was found to be undetectable in all patients studied. Interestingly, however, in vitro studies showed that the number of erythroid progenitor cells (CFU-e, BFU-e) and the response to Epo of these progenitors in even uremia were normal except one patient who had severe secondary hyperparathyroidism. This suggests that bone marrow erythroid progenitor cells of dialyzed patients may be intact in both quality and quantity. However, uremic autologous sera, added to the culture system, produced a dose-related inhibition of erythroid colony formation in vitro. Sera from uremic patients also inhibited erythroid colony formation in normal human bone marrow cultures, suggesting the presence of inhibitor (s) (uremic toxins) in uremic sera. Thus, it is important to emphasize that the existence of serum inhibitor (s) of erythropoiesis should be considered in the treatment of patients with anemia of CRF with erythropoietin in vivo.抄録全体を表示PDF形式でダウンロード (390K) -
—真性赤血球増加症を中心として—前田 秀明, 山田 英雄1985 年 26 巻 5 号 p. 674-684
発行日: 1985年
公開日: 2009/02/02
ジャーナル 認証ありIn the soft agar culture system containing fetal bovine serum, bone marrow cells from polycythemia vera (PV) patients as well as normal subjects gave rise to CFU-E and BFU-E colonies in response to erythropoietin (EPO). In PV, however, the dose response curve of CFU-E colony growth was shifted to the left and many endogenous CFU-E (end-CFU-E) colonis were formed without addition of exogenous EPO in all cases.
Although end-CFU-E colony formation was observed in small portion of cases in normal and other hematological disorders, the ratio of end-CFU-E's to the total CFU-E's was remarkably increased in PV (mean 27.0%), compared to that in other conditions (mean 1.0%).
In addition, enlarged pool size of CFU-E's was formed in PV.
Enhanced colony growth of CFU-E and BFU-E in low oxygen tension cultures was observed in both normal condition and PV. The degree of increased colony growth was greater in low EPO concentrations than in high EPO concentrations. The colony formation of end-CFU-E's in PV was also markedly increased in a hypoxic condition.
Furthermore, cell cycle studies of marrow progenitors revealed that in PV the percentages of progenitors in S-phase were higher in CFU-E, BFU-E and CFU-mix, but normal in CFU-GM. About 90% of end-CFU-E's were in S-phase.
These results indicate that erythroid progenitors in PV proliferate and/or differentiate in a different cell cycle state, and in a different way of response to EPO, while the response of erythroid progenitors to the altered oxygen tensions was maintained like normal subjects.抄録全体を表示PDF形式でダウンロード (819K) -
外山 圭助, 中沢 肇, 青木 功, 岡本 眞一郎, 新保 卓郎, 大島 充一, 吉田 輝彦, 阿部 信子, 宮内 潤, 広橋 説雄, 秦 ...1985 年 26 巻 5 号 p. 685-693
発行日: 1985年
公開日: 2009/02/02
ジャーナル 認証ありErythropoietin (Ep)-producing tumor has been thought to be clinically associated with erythrocytosis. Incidence of erythrocytosis is generally low in renal cancer (0.9∼3.5%), in hepatoma (0∼3.8), in uterine myoma (0.3∼2.2), except in cerebellar hemangioblastoma (11.1∼22.2%) at the Keio University Hospital. However, transplantation of hepatoma and yolksac tumor from the patients without erythrocytosis showed an elevation of hematocrit and increase of Ep in the tumorous tissues in the nude mice. Existance of Ep was immunologically demonstrated in the hepatoma tissue from tumor-bearing nude mice, indicated that this tumor was one of real Ep-producing tumors. Erythropoietic activity was markedly elevated in the cystic fluid of a renal cancer and 2 cerebellar hemangioblastomas unaccompanied by erythrocytosis, while it was not found in that of the other brain tumors. These tumors could be subclinical Ep-producing tumors as the well documented Ep-producing tumors in the kidney and cerebellum have been reported.
The above data suggest that Ep-producing tumor is not necessarily associated with erythrocytosis in the patient and will be more often demonstrated through development of sensitive method specific for Ep measurement.抄録全体を表示PDF形式でダウンロード (786K) -
溝口 秀昭, 山田 英雄1985 年 26 巻 5 号 p. 694
発行日: 1985年
公開日: 2009/02/02
ジャーナル 認証ありPDF形式でダウンロード (55K)
臨床研究
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山崎 博之, 倉石 安庸, 高崎 信子, 尾関 博重, 益頭 尚典, 永峯 檀二郎, 薄井 紀子, 中村 督, 相羽 恵介, 横山 謙三, ...1985 年 26 巻 5 号 p. 695-700
発行日: 1985年
公開日: 2009/02/02
ジャーナル 認証ありForty-nine patients with non-Hodgkin's lymphomas in clinical stages I and II have been treated with an adjuvant chemotherapy consisting of vincristine, cyclophosphamide and prednisolone after radiotherapy or operation. Thirteen cases (27%) have relapsed and the pattern of relapse have been studied.
The results were summarized as follows: 1) Most relapsed cases were diffuse type. 2) Eleven cases (85%) relapsed within 1 year. 3) Majority of them relapsed from extra-irradiated fields, especially the opposite side of diaphragma. 4) Prognosis of the relapsed cases were generally poor with a median survival time of 24 months.抄録全体を表示PDF形式でダウンロード (421K) -
大久保 進, 生間 敬博, 浜口 保武, 安永 幸二郎1985 年 26 巻 5 号 p. 701-708
発行日: 1985年
公開日: 2009/02/02
ジャーナル 認証ありDouble Filtration Plasmapheresis (DFPP), one of the new methods of therapeutic plasmapheresis, was applied to a high-responder of haemophilia A with IgG inhibitor, and its effectiveness as well as its side effects were discussed, as no papers concerning the application of DFPP to haemophiliacs with inhibitors could yet be found in the literature.
Case: H.I., 19 y.o., male, 47 kg body weight. He suffered from intramuscular bleeding in his left calf. As a high-responder of haemophilia A (severe type) showing maximal serum inhibitor level as high as 255 Bethesda Unit/ml, the “bypass therapy” using prothrombin-complex concentrate applied could not significantly improve his haemostatic status. In order to lower his serum inhibitor level, DFPP was put into practice in two successive days, treating his own plasma 2,700 ml and 1,500 ml respectively, resulting in a significant fall of his serum inhibitor level down to 7.9 from 14.0 B.U./ml without any rebound phenomenon. Thereafter, the “bypass therapy” alone could successfully maintain his improved haemostatic condition. As to the side effects of DFPP, only slight hypotension and haemolysis in the circuit tube were recognized, which needed no particular management.
Hereafter, DFPP might become one of the useful means in the management of haemophiliacs with inhibitors.抄録全体を表示PDF形式でダウンロード (591K) -
鈴木 久三, 小嶋 哲人, 佐野 雅之, 斎藤 英彦, 川本 幹夫1985 年 26 巻 5 号 p. 709-716
発行日: 1985年
公開日: 2009/02/02
ジャーナル 認証ありTen adult cases of idiopathic thrombocytopenic purpura (ITP) were treated with oral administration of danazol (400 mg per day). One of 10 cases had not previously treated, but other 9 cases had been treated with either splenectomy, or administration of prednisolone, azathiopurine, cyclophosphamide or high dose intravenous gammaglobulin. Agents, which had been given at the start of the administration of danazol, were continued as same. Platelet counts before treatment were less than 3×104/cmm in all cases.
In 5 of 10 cases, these values increased to 5×104/cmm over 2 months. Regarding to side effects, titers of serum transaminase were elevated in 4 cases, but these elevations were mild and transient. Nausea and menstral disturbance were observed in 2 cases. Consentrations of danazol in blood were found to be 42∼200 ng/ml (mean 118 ng/ml) during treatment. Platelet bound IgG which was measured by the SRID method, was 6.0±4.7 fg/platelet before danazol therapy and 25.7±13.0 fg/platelet after the therapy. C1·INA was 160±31% before and 226±31% after the therapy.抄録全体を表示PDF形式でダウンロード (548K) -
森 眞由美, 在家 裕司, 藤原 睦憲, 森 茂郎1985 年 26 巻 5 号 p. 717-720
発行日: 1985年
公開日: 2009/02/02
ジャーナル 認証ありThis paper described an immuno-peroxidase staining procedure of routin blood smears and smears made by using a cytocentrifuge. A modified avidin-biotin-peroxidase method was introduced on this staining. This method has many advantages. Only 2 ml of blood is enough to stain 24 lymphocyte markers. Smears can be stored for two weeks before staining. Many preparations can be stained at a time. Stained slides can be stored. The most advantageous point is that the morphological features of labelled cells can be observed microscopically. The lymphocyte subpopulations of peripheral blood from 10 normal donors and from 10 aged donors were studied by using this technique. The values of Leu 2a, 3a, 4, 7 positive cells and B cells were 23.5%, 39.4%, 62.3%, 11.7% and 14.7% respectively. In the aged donors, the values of Leu 2a, 3a, 4 positive cells decreased in comparison with the young group.抄録全体を表示PDF形式でダウンロード (374K) -
—Methotrexate大量療法を中心とした多剤併用化学療法—佐々木 邦明, 川井 進, 藤本 孟男, 二宮 恒夫, 菊池 昌弘, 三井 徹次, 楊井 正紀, 駒沢 勝, 岡田 敏夫, 内海 治郎, 三 ...1985 年 26 巻 5 号 p. 721-730
発行日: 1985年
公開日: 2009/02/02
ジャーナル 認証ありTwenty six children with non-Hodgkin's lymphoma (NHL) were treated with multi-drug combination protocols including high-dose methotrexate (50∼200 mg/kg) (H-D-MTX) and citrovorum factor (CF) rescue.
The initial pilot study which was designed to evaluate the efficacy of H-D-MTX against the childhood NHL and was scheduled three administrations of weekly vincristine (VCR) and H-D-MTX (50, 100, 150 mg/kg) as the initial induction therapy, was investigated on seven patients (Stage I+II 3, III+IV 4). Out of seven patients who had sequential consolidation consisted of VCR, adriamysin (ADR), prednisolone (Pred), and H-D-MTX, followed the maintenance therapy with VCR, Pred, MTX (225 mg/m2), mercaptopurine (6MP), and pulse H-D-MTX every 14 weeks, four were achieved in complete remission (CR), and remained in no evidence of the disease for 9+-46+ months.
Five patients in 1981 to 1982 were treated with the protocol for high-risk ALL “CCLSG HR 811 A”, consisting of three-drug (VCR, Pred, cyclophosphamide (1,200 mg/m2) (CPM)) induction, the early consolidation with H-D-MTX and ADR, and the cyclic maintenance with H-D-MTX, VCR, 6MP, and Pred. Out of five patients, three were achieved in CR, and two of three CR patients were survived for 2.6+ and 29+ months without the evidence of the disease.
In the third study, 14 patients were treated with protocol “NHL 8201” consisted of four-drug (VCR, CPM, ADR, Pred) induction, consolidation with H-D-MTX, and cyclic maintenance with VCR, Pred, MTX, 6MP, CPM, ADR, and pulse H-D-MTX every 15 weeks. The CR induction rate was 78.6% and continuous CR rate estimated by Kaplan Mier method was 66.7% at 18-month.
The total CR induction was 69.2% (92% for Stage I+II and 46% for Stage III+IV), and three-year continuous CR rate was 74%. No CNS invasion was observed.
These data suggested that the early consolidation with H-D-MTX was highly effective to prevent the early systemic relapse as well as to prevent the meningeal involvement of childhood NHL.抄録全体を表示PDF形式でダウンロード (731K) -
川井 進, 藤本 孟男, 谷田部 道夫, 三間屋 純一, 小泉 晶一, 太田 茂, 駒沢 勝, 田中 清, 楊井 正紀, 日吉 保彦, 川上 ...1985 年 26 巻 5 号 p. 731-736
発行日: 1985年
公開日: 2009/02/02
ジャーナル 認証ありTwenty children with acute non-lymphocytic leukemia (ANLL) were examined for immunological leukemic cell surface markers. They were registered in Children's Leukemia & Cancer Study Group (CCLSG) from May, 1982, through July, 1984, and treated with Protocol ANLL-811.
Morphological (French-American-British classification) and cytochemical examinations were made by the CCLSG central laboratory of cytology.
Surface antigens of leukemic cells in the bone marrow were analysed by the indirect immunofluorescence method using a series of monoclonal antibodies (Coulter clone: Mo1, Mo2, MY4, MY7, MY9). The expression of myeloid-associated antigens varied as follows MY7 70.0%, MY9 61.1%, MCS-2 68.5%, Mo1 40.0%, Mo2 0.7% and MY4 15.8%.
The patients were classified into four immunological phenotypes according to Griffin's classification (Group I: Ia+, MY4-, MY7+, Mo1-, Group II: Ia+, MY4-, MY7+, Mo1+, Group III: Ia-, MY4-, MY7+, Mo1+, Group IV: Ia+, MY4+, MY7+, Mo1+). Five patients were assigned to Group I, 4 to Group II, 1 to Group III, and 2 to Group IV. The classification couldn't be applied in eight patients.
In a comparison among four immunological subclasses of initial clinical features, white blood cell count, and complete bone marrow remission rate, no significant difference was found.
Further investigation is necessary to determine whether surface marker phenotyping is a more useful predictor of therapeutic response than morphological classification.抄録全体を表示PDF形式でダウンロード (493K) -
吉岡 朗, 亀井 敏昭1985 年 26 巻 5 号 p. 737-742
発行日: 1985年
公開日: 2009/02/02
ジャーナル 認証ありA case of thrombotic thrombocytopenic purpura (TTP) treated sucessfuly by plasmapheresis, anti-platelet drugs and pred nisolone is reported.
A 33 years old housewife was diagnosed TTP because of microangiopathic hemolytic anemia, thrombocytopenia, proteinuria, microhematuria, coma, tonic convulsion and high fever. LDH was 3916 IU/L and all isozyme types were equally elevated. Serologically, anti-nuclear antibody was weakly positive, but other autoantibodies and immune complex were negative. Plasma 6-keto-PGF1α level was as low as 36 pg/ml and TXB2 was 280 pg/ml.
Gum biopsy revealed fibrin thrombus in the lumens of the submucosal capillaries and deposition of hyaline-like substances in the subendothelial regions without any activities of fibrinogen, IgG, A, M or complement in those regions by PAP method.
Treatment was started with pred nisolone 100 mg, Aspirin 1 g, dipyridamole 20 mg, urokinase 24,000 U. Plasmapheresis of 3,000 ml by Hemonetics PEX was done. Platelet count increased rapidly and patient recovered fully without any sequelae. Plasmapheresis is effective method to be chosen firstly in the treatment of TTP.抄録全体を表示PDF形式でダウンロード (576K)
症例
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森 啓, 新倉 春男, 寺田 秀夫, 光谷 俊幸, 佐川 文明, 杉山 喜彦, 風間 和男1985 年 26 巻 5 号 p. 743-749
発行日: 1985年
公開日: 2009/02/02
ジャーナル 認証ありWe reported a case of 34 yrs old man. He was admitted to our hospital with complains of anemia and splenomegaly.
Laboratory findings were as follows: Hb13.1 g/dl Plts 10.5×104/μl, WBC 5,900/μl (tumor cell 2.5%), LDH 1,200 WU, Lysozyme 13.4 μg/ml, IgG 919 mg/dl, IgM 543 mg/dl. Bone marrow finding showed normocellular with 18.2 tumor cells. Cytogenetic study revealed ahypertetraploid type. This case was diagnosed as malignant histiocytosis from morphologic features, cytochemical studies of tumor cells, and clinical course.
After four months from admission, the serum a marked elevation of monoclonal IgM of Kappa type. The tumor cells were strained with IgM florescence antibody technique and might produce immunoglobulin was suggested. The chemotherapy combined with cyclophosphamide, vincristine, ACNU, and prednisolone, and radiation therapy to splenomegaly were ineffective. He died with plumonary bleeding in October 1980. The total survival of this patient was about one year.
Pathological findings were as follows: Systemic enlargement of the lymphnodes and diffuse infiltration of tumor cells into kidney, liver, and spleen.
We discussed the correlation of macrogloblinemia and cytogenetic study in malignant histocytosis.抄録全体を表示PDF形式でダウンロード (866K) -
北野 喜良, 相楽 達男, 小池 清一, 川上 裕隆, 飯島 康美, 鍛治 良一, 今井 俊輔, 斎藤 博, 古田 精市, 安藤 伯秋, 中 ...1985 年 26 巻 5 号 p. 750-755
発行日: 1985年
公開日: 2009/02/02
ジャーナル 認証ありA case of the blast crisis of chronic myelogenous leukemia (CML) with a spontaneous formation of numerous megakaryocyte and basophil colonies is reported.
A 27-year-old man, who had been diagnosed as CML 18 months before, was admitted to our hospital because of high fever and giant splenomegaly. There were undifferentiated blast cells, many basophils and a few micromegakaryocytes in the circulating blood; bone marrow examination showed a number of megaryocytes and myelofibrosis. The blast cells had markers as the following; POX (-), PAS (+), ACP (+), αNBE (-), E-rosette (-), sIg (-), TdT (-). Cytogenetic studies showed 51 XY, -7, +6q-, +8, +17, +19, +22q-, +mar, 3q+, 6q-, 9q+, 22q- karyotype. He responded poorly to DVP therapy and BH-ACDMP therapy, and finally died of the complication of aspergillus pneumonia 46 days after admission.
We examined colony formation of his circulating blood five times according to Iscove's methylcellulose method. A number of megakaryocyte colonies and basophil colonies were observed at day 7 and day 14 of culture. These colony formation also occured spontaneously even with no addition of erythropoietin or medium conditioned by phytohemagglutinin-stimulated leukocytes (PHA-LCM). The chemotherapy resulted in a dramatic decrease of these colony formation.抄録全体を表示PDF形式でダウンロード (668K) -
石山 泰二郎, 杉本 正邦, 若林 芳久, 広瀬 俊一1985 年 26 巻 5 号 p. 756-760
発行日: 1985年
公開日: 2009/02/02
ジャーナル 認証ありA 60-year-old man was admitted to our hospital with epigastralgia and low-grade fever in Oct. 1978. Roentogenologic and endoscopical examinations revealed early gastric cancer. The tumor was surgically resected. In the resected specimen, histopathological examinations confirmed the lesion to be IIc type early gastric cancer without lymph node metastasis.
After operation, the patient has been treated with 5-Fu 600 mg/day for 33 months. In Nov. 1981, a diagnosis of chronic myelogeneous leukemia was made from the hematological findings of peripheral blood, bone marrow and positive Philadelphia chromosome.抄録全体を表示PDF形式でダウンロード (473K) -
重清 俊雄, 白神 〓, 川内 茂徳, 平井 祐二郎, 武市 俊彰, 猪本 享司, 井上 利彦, 新谷 保実, 高田 幸伸, 水口 安則, ...1985 年 26 巻 5 号 p. 761-765
発行日: 1985年
公開日: 2009/02/02
ジャーナル 認証ありDysfibrinogenemia was found in a 70-year-old female with nephrotic syndrome (urinary protein, 6.6 g/day; serum total protein, 3.5 g/dl). Plasma fibrinogen concentration showed different values by several assay methods used; it was decreased to 105 mg/dl by thrombin time method, but increased to 578 mg/dl, 627 mg/dl and 581 mg/dl by micro-Kjeldahl method, tyrosine method and Laurell method, respectively. Plasma fibrinogen levels in 7 family members were almost normal by the four different assay methods.
Purified fibrinogen (200 mg/dl) of the patient gave thrombin time of 188.2 sec (control, 30.8) and reptilase time of over 1800 sec (control, 78.6), both of which were greatly prolonged. Patient's fibrinogen showed normal release of fibrinopeptide and cross-linking by factor XIII, but polymerization of fibrin monomers was impaired. Molecular weight, antigenicity, electrophoretic mobility and carbohydrate content of the patient's fibrinogen did not differ from those of normal fibrinogen. Thus the cause of impaired polymerization in the present fibrinogen requires further study at a molecular level.抄録全体を表示PDF形式でダウンロード (424K) -
安倍 隆, 田中 久夫, 斉藤 和雄, 中澤 眞平, 森 泰二郎, 西野 和良, 杉田 完爾, 高根 恵子, 林 泰秀, 桜井 雅温1985 年 26 巻 5 号 p. 766-771
発行日: 1985年
公開日: 2009/02/02
ジャーナル 認証ありA female newborn with congenital leukemia is reported. The patient was referred to our hospital on the 4th day of life because of hepatosplenomegaly and anemia. Hematological findings on admission revealed remarkable leukocytosis (259,400/mm3) with 94.5% blasts, Hb 7.3 g/dl and platelets 56,000/mm3. Her bone marrow was normocellular with 95.6% blasts as seen in the peripheral blood. Morphology of the blasts stained by Giemsa method was compatible with lymphoid leukemia (FAB, L2 type). The blastic cells showed negative peroxidase stain and negative α-naphthyl butyrate esterase (ANBE) stain. The immunological phenotype was sIg-, E-rosette-, EA-rosette-, EAC-rosette-, Ia like antigen+, common ALL antigen+ and myelomonocytoid antigen+.
The first remission was achieved by VP therapy, however, on the 9th week of disease the monocytoid blastic cells increased in peripheral blood and bone marrow. At the time of relapse, blastic cells disclosed IgG-Fc-receptors, positive peroxidase reaction and strong positive ANBE reaction, which was completely inhibited with NaF. Chromosomal analyses by G— and Q— bands revealed 46, XX, t (11; 19) (q23; p13), and the time of relapse additional abnormalities, +8 and i (19p), were also disclosed. These results might suggest that the blastic cells of this case were compatible with bone marrow precursor cells or myeloid progenitor cells which were proposed by Parkin et al.
The patient died of CNS leukemia and pneumonia at the 7th month of life.抄録全体を表示PDF形式でダウンロード (880K) -
米満 博, 寺田 洋臣, 金沢 正一郎, 近藤 春樹, 内山 幸信, 奥田 邦雄, 重田 英夫, 山口 覚太郎1985 年 26 巻 5 号 p. 772-776
発行日: 1985年
公開日: 2009/02/02
ジャーナル 認証ありA 45-year-old woman was diagnosed as having chronic myelogenous leukemia (CML) in June, 1977. She was successfully treated with busulfan. However, myeloid blast crisis of CML developed 4 years and 7 months after diagnosis. The white blood cell count was 8,700/mm3 with 7% myeloblasts and 57% promyelocytes. The score of neutrophil alkaline phosphatase was 172 by the method of Tomonaga. Cytochemically, the myeloblasts showed negative peroxidase, negative Sudan Black B, slightly positive alpha naphtyl butyrate esterase, slightly positive naphthol AS-D chroloacetate esterase and negative periodic acid Schiff reaction. The patient responded well to the DCMP (daunorubicine, cytosine arabinoside, 6-MP and prednisolone) therapy and BH-AC DMP (behenoyl-Ara C, daunorubicine, 6-MP and prednisolone) therapy. She died two years after the diagnosis of blast crisis.
At autopsy, myeloblastic tumor was seen in the periost of the 10th chest vertebra.抄録全体を表示PDF形式でダウンロード (537K) -
望月 敏弘, 岡崎 俊朗, 石倉 浩人, 和泉 洋一郎, 田嶌 政郎, 澤田 博義, 内野 治人, 藤原 久義1985 年 26 巻 5 号 p. 777-782
発行日: 1985年
公開日: 2009/02/02
ジャーナル 認証ありA 54-year-old male taxi driver with ALL was admitted to our hospital and remission was induced by one course of LAdVP (a combination chemotherapy for ALL, L; 1-Asparaginase, Ad; Adriamycin, V; Vincristine, P; Prednisolone) therapy. After remission for three months, he relapsed and was given one course of AdVEMP (a combination chemotherapy for ALL, Ad; Adriamycin, V; Vincristine, E; Cyclophosphamide, M; Methotrexate, P; Prednisolone) as remission induction therapy without benefit. Then he was administered high dose of cytosine arabinoside (3 g/M2, twice a day, 6 days) and hematological remission was achieved. However he developed an episode of severe congestive heart failure during his nadir period.
This case record reports his clinical coarse and histological findings of the heart and the possible causative role of high dose cytosine arabinoside for congestive heart failure is also discussed.抄録全体を表示PDF形式でダウンロード (546K) -
松本 美富士, 三竹 愛子, 山本 正彦1985 年 26 巻 5 号 p. 783-788
発行日: 1985年
公開日: 2009/02/02
ジャーナル 認証ありSweet's disease (acute febrile neutrophilic dermatosis) is an unusual condition characterized by acute or recurrent episodes of distinctive skin lesions, fever, leukocytosis, a dramatic response to corticoid therapy and a histologic picture of dense dermal infiltration with mature neutrophiles. Recently, it has also been reported in association with malignant neoplasmas. Of approximately 150 cases of Sweet's disease described in the literature, there are 15 cases with acute nonlymphocytic leukemia and one case of chronic myelogenous leukemia.
A case of Sweet's disease is reported in a patient with myelodysplastic syndrome. A 57 year-old man was diagnosed as Sweet's disease by the presence of upper respiratory tract infection, high fever, painful erythema on general body, which was characterized by dense dermal infiltration with mature neutrophiles. Other hematologic findings were normochromic and normocytic anemia, a depressed alkaliphosphatase activities in peripheral neutrophiles and maturation arrest in myeloid series and increased number of monocytic series in bone marrow. He began receiving corticosteroid therapy, and within 24 hours he had a dramatic improvement in above symptoms. In spite of clinical improvement leukopenia with neutropenia was disclosed after corticosteroid therapy. One month later he showed same clinical signs associated with severe leukocytosis with leukemoid reaction and progressive anemia. Leukopenia with neutropenia was showed again after corticosteroid therapy. Clinical episodes of Sweet's disease with leukocytosis appeared periodically once a month, associated with leukopenia and peripheral monocytosis after clinical improvement with corticosteroid therapy. These hematological findings were suggested the presence of myelodysplastic syndrome.
It is a very rare case that Sweet's disease in associated with myelodysplastic syndrome such as our case.抄録全体を表示PDF形式でダウンロード (724K) -
村井 佳代子, 安成 憲一, 安田 光隆, 根来 伸夫, 吉川 智加男, 巽 純子, 巽 典之, 神崎 暁郎, 杉原 尚, 下田 雅大, 橋 ...1985 年 26 巻 5 号 p. 789-794
発行日: 1985年
公開日: 2009/02/02
ジャーナル 認証ありA rare case (70-year-old-female) of congenital stomatocytosis with hemolytic anemia, increased autohemolysis, increased osmotic fragility, decreased red cell deformability and shortened red cell survival is described. Sodium efflux, especially ouabain-insensitive sodium “leak-out”, was markedly increased, although sodium influx was essentially normal. Thus, the red cell sodium content was abnormally low with a normal red cell potassium content. The patient suffered from recurrent blood vessel disorders with pulmonary embolism. The pathogenesis might be related to the decreased red cell deformability in this patient, because no coagulopathy was present.抄録全体を表示PDF形式でダウンロード (717K)
研究会
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1985 年 26 巻 5 号 p. 795-799
発行日: 1985年
公開日: 2009/02/02
ジャーナル 認証ありPDF形式でダウンロード (454K)
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