巻号一覧
22 巻, 12 号
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臨床研究
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倉田 義之, 椿尾 忠博, 林 悟1981 年 22 巻 12 号 p. 1847-1851
発行日: 1981年
公開日: 2009/01/26
ジャーナル 認証ありThe sera of patients with various hematological disorders were tested for their circulating immune complexes (CIC). CIC were detected by solid-phase C1q EIA, anticomplementary activity, platelet aggregation and antiserum to aggregated human IgG. CIC were positive in 10∼60% of patients with leukemia and malignant hematological disorders, 17∼29% of patients with ITP, 0∼36% of patients with aplastic anemia, 13∼44% of patients with collagen disease. Significant correlation was obtained between the results of C1q EIA and anti-complementary activity, but there was no correlation between other methods. It is important to try several methods based on different principle in order to detect CIC.抄録全体を表示PDF形式でダウンロード (366K) -
中西 康雄1981 年 22 巻 12 号 p. 1852-1864
発行日: 1981年
公開日: 2009/01/26
ジャーナル 認証ありAn electron microscopic study of leukemic cells from 5 patients with acute promyelocytic leukemia (APL) revealed two peculiar RER-related features. A honeycomb-like inclusion in RER was observed in all cases. All 5 cases had a collection of RER in a corollaceous pattern. In detail this construction the center of which was a smooth, round membrane, from which several enlarged RER spread out in a radial pattern. Sections made in a longitudinal direction revealed that these enlarged RER ajoined each other in a parallel manner. Of the electron microscopic findings of APL cells, these two patterns are most peculiar and have not yet been found in other leukemic cells.抄録全体を表示PDF形式でダウンロード (4628K) -
森 啓, 萩原 昭二, 西山 博明, 毛利 博, 松野 一彦, 新倉 春男, 寺田 秀夫, 風間 和男1981 年 22 巻 12 号 p. 1865-1874
発行日: 1981年
公開日: 2009/01/26
ジャーナル 認証ありTwenty six patients of aplastic anemia were treated testosterone injection during 1965 to 1980. Among of total cases, fourteen cases (54%) showed a improvement. However, the increase of platelet count was less than those of hemoglobin and white blood cells even in responded cases.
The good response was obtained in the cases with platelet counts more than 2×104/ul before treatment. According to the author's marks adding to hemoglobin value, the number of neutrophils, of platelets and of reticulocytes, the cases with high marks indicated good prognosis. The most cases showing good response had hyperplastic or normoplastic bone marrow, and none without improvement showed hyperactive marrow.
The adverse reaction of long-term injection with testosterone was slight transient increase of GOT and GPT.
The eleven cases died with bleeding of infection.抄録全体を表示PDF形式でダウンロード (855K) -
—Baumgartner法による検討—滝 正志, 稲垣 稔, 富田 幸治, 目黒 嵩, 山田 兼雄1981 年 22 巻 12 号 p. 1875-1879
発行日: 1981年
公開日: 2009/01/26
ジャーナル 認証ありA hemorrhagic tendency has been observed in patients with glycogen storage disease, type I (GSD-I), and their abnormalites have also been reported to become normal after glucose infusion. We studied 3 patients with GSD-I who had mild to severe bleeding tendencies, especially the effect of glucose infusion on their platelet function, coagulation system and other biochemical data. In order to elucidate the bleeding tendencies, we measured the platelet adhesion to the subendothelium and thrombus formation using a special method developed by Baumgartner.
Before the glucose infusion the patients showed hypoglycemia, hyperlipemia, impaired platelet aggregation, mild defective adhesion to subendothelium and impaired thrombus formation. After 10 days of the infusion, platelet aggregation, adhesion and thrombus formation were improved together with the biochemical abnormalities.
The results suggested that the bleeding disorder in GSD-I was an acquired platelet dysfunction which was improved by the infusion of glucose. The result of the study by the Baumgartner method suggested that this platelet dysfunction was caused mainly by a defective release reaction and, in addition, partly by a mild adhesion impairment.抄録全体を表示PDF形式でダウンロード (484K) -
(1) Filtration leukapheresisによる顆粒球の採取とその機能末永 孝生, 尾高 和亮, 塩原 信太郎, 幸道 秀樹, 石野 千津子, 森 孝夫, 服部 絢一, 原田 実根1981 年 22 巻 12 号 p. 1880-1887
発行日: 1981年
公開日: 2009/01/26
ジャーナル 認証ありThe effect of collection procedure of filtration leukapheresis (FL) and in vitro function of FL-granulocytes were studied. Granulocyte collection by FL was carried out for 2 hrs and donors were given 12,000u. of heparin intravenously. Complete blood count done immediatly before and after FL showed that hemoglobin did not change while platelets were decreased by 40,000/μl.
Transient leukocyte decrease was observed early in the procedure and leukocyte count recovered by the end of the procedure.
Functions of FL-collected neutrophils, including trypan blue dye exclusion test, chemotactic activity and in vitro killing of staphyllococcus aureus were studied.
Overall functions of FL-neutrophils were abnormal when studied by these tests. When these FL-neutrophils were separated on the basis of elution time, however, loosely adherent cells eluted with initial tapping the culum showed near-normal functions. In contrast, highly adherent cells eluted by the late tapping were markedly deteriolated in in their functions.
These observations suggest that early-eluted FL cells may be useful for the clinical granulocyte transfusions.抄録全体を表示PDF形式でダウンロード (557K) -
(2) Filtration leukapheresisによる顆粒球輸血の臨床効果について末永 孝生, 尾高 和亮, 塩原 信太郎, 幸道 秀樹, 石野 千津子, 森 孝夫, 服部 絢一, 原田 実根1981 年 22 巻 12 号 p. 1888-1896
発行日: 1981年
公開日: 2009/01/26
ジャーナル 認証ありTo evaluate the therapeutic efficacy of granulocyte transfusions, 49 patients with profound granulocytopenia (<200/mm3) showing no response to antibiotic therapy were studied in 60 febrile episodes. We comparec 27 transfused patients for their 20-day survival with 22 untransfused patients. The transfused patients received 131 transfusions of FL-granulocytes in 29 febrile episodes. These two groups of patients were comparable in terms of age, sex, disease, and hematological state.
Twenty transfused patients survived from 31 febrile episodes while 15 untranufused patients from 29 febrile episodes.
The result is analysed on the basis of bone marrow recovery which indicates a granulocyte count more than 500/mm3 on day 20 post-transfusion. In patients without marrow recovery, 7 of 18 (38.9%) transfused patients survived whereas only 2 of 15 (13.3%) untransfused did so. These different survival rates are not significant statistically. In contrast, both groups of patients with bone marrow recovery showed extremely high survival rates; 100% for the transfused patients and 92.9% for the untransfused.
These observations suggest that all seriously infected patients may not benefit from granulocyte transfusions and its therapeutic effect may be dependent on bone marrow recovery of the patients so treated.抄録全体を表示PDF形式でダウンロード (645K) -
土屋 達行, 熊坂 一成, 河野 均也, 大島 年照, 桑島 実1981 年 22 巻 12 号 p. 1897-1908
発行日: 1981年
公開日: 2009/01/26
ジャーナル 認証ありThe French-American-British (FAB) Co-operative Group has developed proposals for the classification of the acute leukemias (the FAB classification) which should permit a more uniform system of classification and more accurate recording of the distribution of cases entered into clinical trials.
Since 1978, this classification has been accepted and is still in use in our University Hospital.
From June 9th to August 5th 1980, according to this classification, a panel of four hematologists (A, B, C and D) had examined bone marrow smears from 70 patients with acute leukemia or disorders that might be confused with acute leukemia.
Reproducibility of diagnosis by one of the panelist “A” who has been examining bone marrow smears in our laboratory was satisfactory (90.3% reproducibility). But complete agreement among the observers was poor (39 out of 70 cases, 55.7%, for A, B and C: 8 out of 23 cases, 34.8%, for A, B, C and D)
These observations suggest that the diagnostic criteria of the FAB classification may not be applied uniformly within the panel.
After the first trial, the four panelists had a meeting to reconsider the criteria of the FAB classification, and a discussion on Wright-Giemsa stained films from 24 selected cases was made to make accurate recording of the cases.
After this meeting, from September 16th to November 8th 1980, a similar test was carried out on 46 cases excluding dysmyelotoietic syndrome (DMPS).
Complete agreement become higher in the second test (39 out of 46 cases, 84.8% for A, B and C: 31 out of 46 cases, 67.4% for A, B, C, and D).
We concluded that recent attempts to define the types of acute leukemia by the FAB classification have been welcomed, but it is still difficult to get a high degree of diagnostic agreement for patients with acute leukemia form morphological findings alone. It was also confirmed from this study that a few of the cases of acute leukemia examined in Japan were not considered to be compatible with any types of acute leukemia described in the FAB classification.抄録全体を表示PDF形式でダウンロード (925K) -
星 順隆, F. Leonard JOHNSON1981 年 22 巻 12 号 p. 1909-1915
発行日: 1981年
公開日: 2009/01/26
ジャーナル 認証ありSixteen children with ALL, ANLL and A. A. have been treated with allogeneic bone marrow transplantation at Children's Orthopedic Hospital and Medical Center (COH & MC) in Seattle from May to November in 1980.
All patients recieved marrow grafts. Eleven patients (69%) survived over 4 to 10 months after marrow transplantation and nine of them (56%) have no troubles until April in 1981.
Sixty three percent of patients had GVHD and 3 patients (19%) died with severe GVHD.
In COH & MC we did not use bioclean room, sterilization of food nor granulocyte transfusion as a prophylaxis of infection. Infections after engraftment occurred in 6 patients (38%). Four out of these patients were recovered, but two were fatel.
As supported cares, all patients have been get platelet transfusions, packed red blood cell transfusions were done in 12 patients and granulocyte transfusions in 2 patients, respectively.
Recent experiences of bone marrow transplantation at COH & MC were analyzed and progress in future as discused.抄録全体を表示PDF形式でダウンロード (532K) -
椿 和央, 岩永 隆行, 入交 清博, 堀内 篤1981 年 22 巻 12 号 p. 1916-1922
発行日: 1981年
公開日: 2009/01/26
ジャーナル 認証ありThe study of cell kinetics has contributed to the understanding of cellular proliferation and antineoplastic drug action in acute leukemia. We have studied the cell kinetics by the flow cytometry for 20 patients of acute non-lymphocytic leukemia. The proliferative compartment (P. C.: S phase and G2+M phase) which were obtained from DNA histogram by the flow cytometry were used as a parameter of proliferation kinetics of leukemic cells. The P. C. size of untreated patients were 9.0±2.8% in AML (n=13), 6.6±0.9% in APL (n=4), 8.2±2.8% in AMMoL (n=2) and 16.1% in AMoL respectively. There were significant correlation between the P.C. size and mitotic index of the bone marrow leukemic cells.
No close relationship were observed between remission and failure cases in the P.C. size.
The P.C. size of pretreatment correlated positively with degree of cytoreduction in the bone marrow leukemic cells during the first 10 days of induction treatment, especially the patients with less than 7% of the P.C. size had lower cytoreductive rate and required long period until complete remission. The increase of the P.C. size before two step in the DCMP two step therapy were about 6%.
From the results described above, the proliferation kinetics of leukemic cells and effect of treatment in acute leukemia were discussed.抄録全体を表示PDF形式でダウンロード (547K) -
河 敬世, 多和 昭雄, 池田 輝生, 朱 欽明, 岡田 正, 金 昌雄, 薮内 百治1981 年 22 巻 12 号 p. 1923-1929
発行日: 1981年
公開日: 2009/01/26
ジャーナル 認証ありIntravenous hyperalimentation (IVH) combined with anticancer therapy has been used to treat 34 children with leukemia and a variety of solid tumor in the past 7 years. The patient's ages ranged from 2 months old to 14 years old (median age; 3 years and 7 months old). The median duration of IVH was 32 days (from 6 to 235 days). Without IVH, we were often interrupted our treatment schedules by various complications depending on chemotherapy and radiotherapy. But with IVH, we were able to replete and support the patient's nutritional state and make them more tolerated to those anticancer treatments. Therefore the chemotherapy of both induction phase and consolidation phase was well performed, and it resulted in prolongation of their survival time.
Catheter sepsis, one of the most serious complications with IVH, developed 8% of them (in general, the rate of occurrence is 10 to 15%). Some patients under myelosuppressive condition and/or opportunistic lung infections were successfully treated with IVH. We thought IVH would be one of the important supportive therapeutic procedures in the treatment of refractory cancer patients.抄録全体を表示PDF形式でダウンロード (510K)
症例
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上瀬 英彦, 麦島 秀雄, 三間屋 純一1981 年 22 巻 12 号 p. 1930-1937
発行日: 1981年
公開日: 2009/01/26
ジャーナル 認証ありA 8-year-old girl of von Willebrand's disease with abnormal factor IX was reported. She repeated episodes of ecchymoses on legs and epistaxis since one year old. Screening coagulation study on admission revealed prolonged bleeding time and activated partial thromboplastin time, but platelet count and prothrombin time were normal. Platelet retention (13%), RIPA (1%), factor IX activity (6∼56%), factor VIII-related antigen (27∼50%) and von Willebrand's factor (0∼6%) were decreased.
The pattern of crossed antigen-antibody electrophoresis of factor VIII showed that the distance of migration was normal, but the peak had more anodal electrophoretic mobility. The pattern of crossed electrophoresis of factor IX showed that the peak was lower, but the distance of migration was normal. Factor IX activity markedly increased after infusion of AHF-MIDORI 2 vials. Bleeding time, platelet retention and RIPA were also improved. Therefore, infusion study of AHF suggested the existence of factor-IX stimulator in AHF.
In family study, mother and younger brother have the same hemorrhagic tendency and these findings indicated that this was a case of von Willebrand's disease with abnormal factor IX.抄録全体を表示PDF形式でダウンロード (1158K) -
恒松 由記子, 小出 亮, 佐々木 道子, 内海 治郎1981 年 22 巻 12 号 p. 1938-1947
発行日: 1981年
公開日: 2009/01/26
ジャーナル 認証ありA family is described in which four children developed ALL. Cytogenetic and immunogenetic studies detect two possible phenotypic markers which predict predisposition to leukemia.
In April 1978, the proband, 2 years old girl developed ALL and admitted to the National Children's Hospital in Tokyo. Her eldest brother and second brother died of ALL consecutively in 1969, 1970 at 3 and 2 years of age respectively. In June 1978, paternal half first cousin of the proband, 3 years old boy, admitted to the Niigata Cancer center with a diagnosis of ALL.
There were no contacts between these two branches of this family, which did not reveal any consanguineous mating. This aggregation of leukemia cases likely resulted from genetic mechanism with a dominant mode of inheritance for genetic susceptibility. Cytogenetic studies of this family showed no constitutional chromosome abnormality nor chromosomal aberration of leukemic cells. But G-banding analysis of cultured skin fibroblasts revealed chromosomal rearrangements in the patients and the obligate carriers.
Furthermore, studies of HLA typing were suggestive to associate certain haplotype with the occurence of leukemia, since the surviving leukemic patients and obligate carriers had the same haplotype AW19 BW40.
These two findings are important as possible phenotypic markers which detect predisposition to leukemia.抄録全体を表示PDF形式でダウンロード (782K) -
藤原 克彦, 松村 隆文, 田中 輝房, 東道 伸二郎, 生田 康治, 新川 正治, 今宿 晋作1981 年 22 巻 12 号 p. 1948-1954
発行日: 1981年
公開日: 2009/01/26
ジャーナル 認証ありA case of congenital pure red cell anemia is reported. At the age of 3 months, the female infant was first noted to have anemia, poor weight gain and several anomalies, such as left blephaloptosis, thenar defect, syndactyly, mesocardia and right radial arterial defect.
Thereafter, the patient has been treated with intermittent blood transfusions, anabolic steroid, ACTH and glucocorticoid over a period of 8 years.
Presently, at 8 years of age, the patient is free of anemia maintained by a minimal dose of glucocorticoid, and she has a normal height (-1.6 SD) and has developed well physically as well as mentally with an I.Q. of 96.抄録全体を表示PDF形式でダウンロード (632K) -
沢田 賢一, 小梁川 義則, 出野 正孝, 上村 康二, 佐藤 雅寛男, 西川 哲裕, 桜間 照喜, 中川 昌一, 安河内 太郎, 中里 堯 ...1981 年 22 巻 12 号 p. 1955-1961
発行日: 1981年
公開日: 2009/01/26
ジャーナル 認証ありA 54-year-old female had been hospitalized because of anemia for six months before admission to Hokkaido University Hospital on January 31, 1978. Physical examinations on admission showed hepatomegaly without splenomegaly. No thymoma was found on chest X-ray film. Her peripheral blood count was as follows: Hb 7.7g/dl, RBC 259×104/cmm, reticulocytes 0‰, WBC 4,400/cmm (neutrophils 10, eosinophils 1, basophils 1, lymphocytes 80, monocytes 8%), platelets 16.5×104/cmm. The marrow aspirate was of normal cellularity but showed marked depletion of erythroblastic series.
She was treated with blood transfusion, corticosteroids, vitamins and anabolic steroids for twenty-one months. Because of the lack of response to these treatments, splenectomy was performed on December 20, 1979. After splenectomy hematological remission was obtained, lasting for over nineteen months.
In vitro colony assay was carried out to clarify whether the patient's serum and/or spleen cells could suppress erythropoiesis. Neither her serum nor spleen cells suppressed CFU-E colony formation from her own marrow cells in remission.
Splenectomy may be a useful tool for treatment of steroid-resistant cases of pure red cell aplasia, as it occurred in the present case.抄録全体を表示PDF形式でダウンロード (452K) -
—臨床像および膜マーカーについて—草刈 章, 中沢 真平, 高根 恵子, 山田 兼雄1981 年 22 巻 12 号 p. 1962-1970
発行日: 1981年
公開日: 2009/01/26
ジャーナル 認証ありTwo patients with B-cell acute lymphoblastic leukemia are reported.
Patient 1: A 3 year and 5 month old boy was admitted to the hospital because of marked enlargement of the both kidneys associated with hyperuricemia (25.2 mg/dl) and elevated serum creatinine (3.3 mg/dl). Bone marrow was replaced almost completly by the blast cells with deeply basophilic cytoplasm and several cytoplasmic vacuoles on the Wright-Giemsa stain. Prednisolone and vincristine were initiated. Despite hyperkalemia (9.6 mEq/L) leading to transient cadiac arrest on the 2nd day of the treatment, a complete remission was achieved. Following 4 weeks remission, he developed meningeal and testicular leukemia, and expired with refractory hematological relapse in five and a half months.
Patient 2: A 3 year and 3 month old boy was admitted with chief complaints of headache, abdominal pain and soreness of the right thigh. Bone marrow aspiration showed a massive infiltration of the blast cells with an identical morphological feature as case 1. Lumbar puncture disclosed meningeal leukemia. Prednisolone and vincristine were administered, but were discontinued because of ileo-ileal interssusception followed by diffuse peritonitis occuring on the 1st day of the therapy. In 3 weeks, he was found to be in complete remission, but in 5 weeks he developed refractory hematological relapse. He died of pneumonia in three and a half months.
Cell surface marker analysis of the lymphoblasts from the bone marrow of both patients disclosed the pattern of B-lymphoid cells in terms of surface immunoglobulin (μ) and B-antigen. In addition to these markers, common ALL antigen was positive on the lymphoblasts from both patients.
The clinical and cytological features of these patients are compatible with notion that B-cell ALL and non-endemic Burkitt's lymphoma may share a common underlying pathogenesis.抄録全体を表示PDF形式でダウンロード (856K) -
佐藤 雄二, 泉二 登志子, 山田 修, 伊藤 弥生, 片平 潤一, 高橋 正知, 水谷 良子, 戸塚 恭一, 溝口 秀昭, 岡田 美智子1981 年 22 巻 12 号 p. 1971-1976
発行日: 1981年
公開日: 2009/01/26
ジャーナル 認証ありA 57-year-old male had noted dermal photosensitivity and shortness of breath 1 year before admission. Since then he had been treated with iron or vitamin B6 in other hospitals but the symptoms did not improve. In July 1980, he was admitted to our hospital for precise evaluation. Physical examinations revealed slight anemia and atrophy of the skin at the area exposed to sun light. Peripheral blood examinations showed microcytic hypochromic anemia with marked poikilocytosis and dimorphism. Bone marrow smears showed erythroid hyperplasia with increase in the number of ringed sideroblasts. From these findings, he was diagnosed as having acquired sideroblastic anemia. Since protoporphyrin in red blood cells increased markedly, dermal photosensitivity was caused by similar mechanisms as erythropoietic protoporphyria. Moreover, chromosomal abnormalities of the marrow cells were noted. Granulocyte-macrophage colony formation and erythroid colony formation were markedly suppressed. From these findings, it can be considered that both sideroblastic anemia and increased levels of protoporphyrin in red blood cells occurred secondary to mutation of the hemopoietic stem cells.抄録全体を表示PDF形式でダウンロード (512K) -
西田 雅喜, 岩永 隆行, 入交 清博, 堀内 篤, 橋本 重夫, 古田 格1981 年 22 巻 12 号 p. 1977-1983
発行日: 1981年
公開日: 2009/01/26
ジャーナル 認証ありA case of immunoblastic lymphadenopathy (IBL) with interstitial pneumonitis and pancytopenia was reported.
A 63-year-old male was admitted to our clinic with severe anemia and generalized lymphadenopathy. Laboratory findings were at follows: Hb 4.3 g/dl WBC 1,300/mm3, Platelets 4.9×104/mm3, Reticulocytes 0‰, CHA 1: 512, a positive Coombs test, hypocomplementemia and polyclonal hypergammaglobulinemia in immunoelectrophoresis. Biopsy specimen of the cervical lymph node showed diffuse proliferation of large lymphocytes (immunoblasts), abundunce of arbolizing small vessels and deposition of amorphous PAS-positive interstitial materials.
Although administration of prednisolone and blood transfusion resulted a partial remission, he died two months later.
Postmortem examination revealed hemosiderosis in liver and spleen, and a generalized fungal infection.抄録全体を表示PDF形式でダウンロード (873K) -
—IgD型PCLにて発症した例とIgG多発性骨髄腫の経過中PCLとなった例—加藤 雅子, 高槻 義夫, 山内 幹雄, 高 誠信, 荒井 一歩, 木我 豊, 山口 了三, 間崎 民夫, 梅田 正法, 白井 達男1981 年 22 巻 12 号 p. 1984-1993
発行日: 1981年
公開日: 2009/01/26
ジャーナル 認証ありTwo cases of plasma cell leukemia (PCL) are reported. The first case was 52-year-old male (Case K. K.). He was admitted to Toho University Hospital on April 10, 1980, because of abnormal peripheral blood picture and anemia. Hepatosplenomegaly, mild anemia, thrombocytopenia and petechiae were present. The WBC count was 10,400/cmm, with 66% of plasma cells. Some of plasma cells showed lymphoid configuration, but possessed surface IgDλ. Serum IgD level was as high as 1,300 mg/dl and Bence Jones proteinuria was present. The diagnosis of IgD PCL was made, and he received the intensive combination chemotherapy of ADM, VCR, CPM and PSL. Notable improvement was obtained after the chemotherapy, but severe hypogammaglobulinemia developed gradually. He died of sepsis at the 7th month from the time of diagnosis.
The second case was 76-year-old male (Case A. I.) with IgG multiple myeloma, who had been successfully treated with cyclophosphamide and β-methason. As his conditions deteriorated at the terminal stage of myeloma, many plasma cells (80.5%) appeared in the peripheral blood. He was diagnosed as PCL and died of cardiac failure. At autopsy, diffuse and extensive infiltrations of plasma cells into multiple organs were detected. It was suggested that PCL should be treated with intensive combination chemotherapy.抄録全体を表示PDF形式でダウンロード (1354K) -
杉田 憲一, 岩間 義彦, 水島 和一郎, 加藤 邦重, 海野 健, 江口 光興, 古川 利温1981 年 22 巻 12 号 p. 1994-2000
発行日: 1981年
公開日: 2009/01/26
ジャーナル 認証ありClinical pictures and cytological and ultrastructual features of a 6-year-old girl with Pre-B cell type acute lymphoblastic leukemia were reported. Her initial clinical presentation was severe anemia, marked leukocytosis, hepatomegaly, mediastinal enlargement Hydronephrosis due to lower urinary truct obstruction by the tumor developed at the time of relapse.
IgM was faintly detectable in cytoplasma of leukemic cells but surface lg was not demonstrable. Abnormal karyotype with marker chromosomes was found (46 XX, 8-, 11-, m+1, m+2,).
Respose to chomotherapy was rather poor, althogh complete remission was easily induced with vincristine, predonisolone and L-asparaginase, and maintained for 3 months. Thereafter, only partial remission and clinical inprovement were obtained with methotrexate, vincristine, cytosine arabinoside and adrimycin and she has been surviving for 18 months from the onset to the time of this writing.抄録全体を表示PDF形式でダウンロード (2236K) -
中村 治雄, 藤本 健一, 中澤 正樹, 阿部 帥, 長谷川 鎮雄1981 年 22 巻 12 号 p. 2001-2008
発行日: 1981年
公開日: 2009/01/26
ジャーナル 認証ありA family with decreased concentration of plasma antithrombin III (AT III) was described.
Propositus was 46-year-old man, who was found chronic pulmonary embolism. His chief complaints were chest pain on the right and dyspnea. In his father and brother, plasma AT III concentration was also decreased. His uncle was died of mesenteric vein thrombosis at 40 years old.
In September 1980, he visited practitioner for shortness of breath and cough. Enlargement of the pulmonary arteries was pointed out on the chest X ray at the clinic. The symptoms, however, gradually improved without treatment. On Dec. 18, he was admitted to the University of Tsukuba hospital for cough, fever, chest pain and difficulty in breathing. Physical examination on admission was negative for the presence of vascular murmur consistent with cardiac systole on the lower lobe of the left lung. Pulmonary perfusion scintiphotography disclosed extensive defect of blood flow in the right lung.
The concentration and activity of AT III in the patient's plasma were proved to be decreased by immunological and biological assay respectively. Other coagulation tests such as prothrombin time, activated partial thromboplastin time, plasma fibrinogen, serum fibrin degradation product, euglobulin lysis time, platelet count and platelet aggregation induced by ADP, epinephrine, collagen and ristocetin were all normal. Although pulmonary embolism improved, the concentration of AT III in plasma remained low.
From these findings a diagnosis of familial AT III deficiency was made.
The pathophysiology of familial AT III deficiency was discussed.抄録全体を表示PDF形式でダウンロード (611K) -
別所 文雄, 藤生 道子, 水谷 修紀, 賀来 秀文, 林 泰秀1981 年 22 巻 12 号 p. 2009-2015
発行日: 1981年
公開日: 2009/01/26
ジャーナル 認証ありElectron microscopic study of two cases of Microgranular acute promyelocytic leukemia was performed. Leukemic cells of both cases had various ultrastructural characteristics which had been reported in cases of typical acute promyelocytic leukemia (APL), except for unusually small granules which measured 100 to 200 mμ in diameter. Among features characteristic for typical APL was there well-developed rough endoplasmic reticulum which was occasionally arranged in corollaceous pattern. There was also peculiar thread-like structure extending from granules and Auer rods. This structure formed circular or concentric profiles and had been reported as circular or concentric profiles of membrane bound dense material in previous literature. Both structures were positive for peroxidase reaction.抄録全体を表示PDF形式でダウンロード (2104K) -
高橋 隆一, 宮入 誠, 村山 直弘, 青木 誠, 福井谷 祐一, 川戸 正文, 伊藤 宗元, 日野 理彦1981 年 22 巻 12 号 p. 2016-2022
発行日: 1981年
公開日: 2009/01/26
ジャーナル 認証ありIncreasing frequency of cases with malignant lymphomas following autoimmune diseases and experimental studies on New Zealand black mice suggest an immunological relationship between autoimmune diseases and the development of malignant lymphomas. We report a case of malignant lymphoma occurring subsequent to autoimmune hemolytic anemia.
Case T.O., a 60-year-old housewife, was first seen at the Second Tokyo National Hospital in June 1979, complaining of general fatigue and exertional palpitation. Examinations revealed anemia, marked reticulocytosis, erythroid hyperplasia of the bone marrow, diminished serum haptoglobin and positive direct and indirect antiglobulin tests. Neither splenomegaly nor lymphadenopathy was found. Anemia was recovered by corticosteroid therapy. In September 1979, when she suffered from herpes zoster, the indirect antiglobulin test became negative. In July 1980, she noticed the swelling of her left lower jaw and biopsy revealed malignant lymphoma of diffuse mixed type. After radiation the swelling subsided and she is now well.抄録全体を表示PDF形式でダウンロード (621K) -
—骨髄血ヘム合成酵素活性の低下—金子 兼三, 真田 雅好, 今泉 信作, 小池 正, 宮下 正弘, 柴田 昭1981 年 22 巻 12 号 p. 2023-2033
発行日: 1981年
公開日: 2009/01/26
ジャーナル 認証ありTwo unrelated patients with erythropoietic protoporphyria (EPP) were studied. Patient R. A. was a 10-year-old girl and patient S. O. was a 51-year-old man.
Diagnosis of EPP was established in both patients by a history of photosensitivity on exposed areas of the skin since childhood and by marked increase of protoporphyrin levels in erythrocytes, plasma and feces.
Heme synthetase activity in erythroblasts was remarkably decreased in both patients, 1.2 and 1.3mμ moles hemin formed/120min./108 erythroblasts, as compared with control values (range 2.5∼6.4; n=5). This metabolic abnormality provided one biochemical explanation for the increased level of erythrocyte protoporphyrin in EPP.
Subnormal levels of Hb without sideropenia were observed in both patients. Ringed sideroblasts were found in 1 and 8%, respectively and by transmission electron microscopy iron deposits in the cristae of mitochondria in erythroblasts were observed, although the ferrokinetic study in patient S. O. was within normal limits.
In patient S. O., the liver was palpable three fingers breadth on admission and intermittent elevations in serum GOT, GPT and γGTP levels were observed. A percutaneous liver biopsy specimen revealed the feature of non-specific chronic hepatitis. Furthermore in frozen, unfixed sections of the liver, brilliant red fluorescence showing the presence of protoporphyrin was evident in a part of hepatocytes and Kupffer cells. After 4 years, he died of hepatic cirrhosis with severe jaundice and hepatic failure.
The oral administration of 15 to 30mg/day of β-carotene was safe and effective for ameliorating the photosensitivity in both patients.抄録全体を表示PDF形式でダウンロード (2126K)
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1981 年 22 巻 12 号 p. 2034-2038
発行日: 1981年
公開日: 2009/01/26
ジャーナル 認証ありPDF形式でダウンロード (450K)
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