臨床血液
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
21 巻, 12 号
選択された号の論文の17件中1~17を表示しています
臨床研究
  • 花田 尚, 依田 安弘, 中澤 正樹, 阿部 帥
    1980 年 21 巻 12 号 p. 1853-1857
    発行日: 1980年
    公開日: 2009/01/26
    ジャーナル 認証あり
    This study was arranged to determine whether serum ferritin level was useful to evaluate an improvement of iron store by oral iron therapy in patients with iron deficiency anemia (IDA). Serum ferritin level, transferrin saturation and bone marrow iron deposit in 12 patients with IDA were studied before the treatment, on improvement of the anemia and after the treatment. Serum ferritin level and transferrin saturation increased significantly along with the course of treatment and the former showed a positive correlation with the latter (r=0.73, p<0.001). After the treatment bone marrow iron appeared to be normal in all cases, but on improvement of the anemia no iron deposit was found in any cases. 31 cases of bone marrow study were performed altogether and were devided into two groups; normal bone marrow iron group and depleted group. Serum ferritin levels of the latter were apparently dissociated from those of the former at the boundary level of approximately 20ng/ml, although transferrin saturation of the several cases in both groups overlapped each other. These findings suggest that serum ferritin level reflects bone marrow iron deposit more accurately than transferrin saturation does. It was considered that serum ferritin level is clinically useful to evaluate the improvement of bone marrow iron store by oral iron therapy.
  • 吉田 幸二, 安藤 精章, 樋口 富彦
    1980 年 21 巻 12 号 p. 1858-1862
    発行日: 1980年
    公開日: 2009/01/26
    ジャーナル 認証あり
    By using coil planet centrifuge system, and Couter counter model ZBI and channelyzer, hemolysis end points (HEP) and mean cell volume (MCV) of erythrocytes were studied in patients with iron deficiency anemia, liver diseases, and advanced gastric cancer to determine whether the apparent association between HEP and MCV might be present. There was a positive correlation between HEP and MCV in patients with iron deficiency anemia. On the other hand, there was a negative correlation between them in patients with liver diseases. However, there was no significant correlation between them in patients with advanced gastric cancer. A significant positive correlation was observed between HEP and either free cholesterol or cholesterol/phospholipid in them. A significant negative correlation was also observed betweem HEP and fibrinogen. Furthermore, there was a significant positive correlation between MCV and serum Fe. The shift of HEP might be closely correlated to the changes of MCV. However, the shift of HEP was not always due to the changes of MCV, and many mechanisms to induce the change should be sought.
  • 郡山 健治, 有森 茂
    1980 年 21 巻 12 号 p. 1863-1868
    発行日: 1980年
    公開日: 2009/01/26
    ジャーナル 認証あり
    The quantitative alteration of T cell subsets was studied on peripheral blood lymphocytes from 20 patients with systemic lupus erythematosus (SLE) and compared with those of 25 healthy adults. Four patients were clinically inactive and 16 were active SLE. Six patients with active SLE were not treated before the study. T Lymphocytes were enumerated as rosette formed cells with sheep erythrocytes. Tγ and Tμ cells were detected by using rosette technique with bovine erythrocytes coated either with IgG or IgM antibodies.
    The proportion and absolute count of T cells were significantly decreased in SLE (p<0.01). Moreover, the total number and percentage of Tγ cells were remarkably reduced (p<0.001). Although the absolute number of Tμ cells significantly diminished (p<0.01), the proportion remained within the control range. The total count of T non γ non μ cells, which are T cells not possessing Fc receptors for IgG and IgM on their cell surface, were not significant between SLE patients and healthy controls. However, the percentage of T non γ non μ cells was higher than control (p<0.05). These disproportions of T cell subsets were not statistically significant between active and inactive SLE. The ratio of Tγ/Tμ cells, however, was significantly lower in active SLE (p<0.01).
症例
  • 山村 真由美, 吉田 喬, 中村 忍, 服部 絢一, 谷本 一夫
    1980 年 21 巻 12 号 p. 1869-1876
    発行日: 1980年
    公開日: 2009/01/26
    ジャーナル 認証あり
    A 56-year-old female was admitted on May 2, 1979 for a persistent high fever, eczema and left exophthalmus of one month duration. On physical examination, left exophthalmus with chemosis and erythematous nodules of about 5 cm in diameter scattered through the trunk were noted, but neither superficial lymphadenopathy nor hepatosplenomegaly was seen. Blood counting showed mild pancytopenia with a few histiocyte-like cells with erythrophagocytosis. These atypical cells, also were found in a small number on the bone marrow smears. Skin biopsy revealed proliferation of atypical cells within capillaries and blood vessels of the skin. Morphological study showed that such atypical cells in the blood, bone marrow and the skin belonged to histiocytes, some of which were immature in dominancy of the nucleus with prominent nucleoli, but many of them were mature in erythrophagocytosis in their huge cytoplasm.
    From the above-mentioned clinical manifestations and laboratory data, a diagnosis of malignant histiocytosis (Rappaport) was made. The patient was treated with combination therapy of vincristine and corticosteroids with a very transient effect, deteriorated rapidly, and died on June 11, 1979, two months after onset. Permission of autopsy was not obtained.
  • 向山 秀樹, 月本 一郎
    1980 年 21 巻 12 号 p. 1877-1884
    発行日: 1980年
    公開日: 2009/01/26
    ジャーナル 認証あり
    Four cases with congenital factor XI (PTA) deficiency from a Korean family were reported. An 11 year old girl with nasal bleeding was found to have prolonged whole blood clotting time and plasma recalcification time, abnormal prothrombin consumption test and abnormal thromboplastin generation test.
    The factor XI activity examined using PTA deficient plasma (DADE) as a substrate was around 20%. Other plasmatic clotting factors were within normal range. The patient's father, mother and younger sister (7 year old) showed 36, 44 and 32% of factor XI activity, respectively. All of them have had mild hemorrhagic tendencies such as nasal and gingival bleeding.
    With these laboratory findings, four patients were diagnosed to be factor XI deficiency, and were classified as a minor deficiency type under Rapaport's criteria.
    Following a single fresh plasma infusion of 5 U/kg of body weight, the factor XI activity was elevated from 23% to 44% and returned to the preinfusion level within 24 hours. But plasma recalcification time and activated partial thromboplastin time were kept normal for 48 to 72 hours after infusion.
  • —PiMの変異型PiMnumazuについて—
    大谷 英樹, 船渡 忠男, 斎藤 正行, 富田 友幸, 杼窪 豊, 和田 薫
    1980 年 21 巻 12 号 p. 1885-1891
    発行日: 1980年
    公開日: 2009/01/26
    ジャーナル 認証あり
    A 56-year-old male with severe α1-antitrypsin deficiency and four cases in his family are reported. The patient (propositus) was admitted because of dyspnea. X-ray films of the chest disclosed marked emphysema. Respiratory examinations revealed increase of residual volume/total lung capacity ratio and forced expiratory volume, and decrease of CO diffusion.
    Cellulose acetate electrophoresis revealed decrease of α1-fraction. Serum α1-antitrypsin was 10 mg/dl by single radial immunodiffusion. Trypsin inhibitory capacity also markedly decreased. Immunoelectrophoresis showed no precipitin line of α1-antitrypsin developed with anti-α1-antitrypsin.
    The α1-antitrypsin phenotype of the patient was designated as a variant of PiM, PiMnumazu, by cellulose acetate isoelectric focusing followed by crossed immunoelectrophoresis, which was more sensitive than acid starch gel electrophoresis and useful for distinguishing the genetic variability of α1-antitrypsin deficiency.
    Family study revealed that the parents, the youngest brother, one of his uncles and one of his aunts were heterozygous for α1-anititrypsin gene. They had no pulmonary emphysema except for the propositus.
  • 島崎 千尋, 中田 哲雄, 西尾 晃, 春山 春枝, 香月 昭人, 中川 雅夫, 杉島 聖章, 伊地知 浜夫, 伊勢村 卓司
    1980 年 21 巻 12 号 p. 1892-1897
    発行日: 1980年
    公開日: 2009/01/26
    ジャーナル 認証あり
    This paper presents a case of T-cell lymphoma with a long survival achieved by radiation and immunochemotherapy.
    A 46 year old male, a non-native of Kyushu district, was admitted to our service for the further evaluation and treatment of subcutaneous tumor of r. shoulder and l. arm on January 1979. Two years prior to admission the patient was diagnosed as malignant lymphoma from the histological findings of dissected nasal septal tumor, which was treated by irradiation therapy alone. A tumor of the r. inguinal region was extripated for further evaluation and was diagnosed as poorly differentiated malignant lymphoma. These tumor cells formed rosette with sheep erythrocytes. Bone marrow aspiration revealed hypocellularity without tumor cells and he was treated by prednisolone alone with poor response and additional local administration of OK-432 was started into the tumor which became soften in consistency and decreased in size. Positive direct Coomb's test and antiplatelet antibody were detected in short time of thrombocytopenia, and the increased administration of prednisolone improved the platelet counts. In spite of immunochemotherapy including prednisolone, Bleomycin, oil-Bleo and OK-432, the tumor size was increased and symptom was aggravated and expired on March 1979 due to shock induced by oil-Bleo injection.
    Through the clinical evaluation of this patient, it is emphasized that 28 months long survival was achieved responding to the treatment with irradiation and local OK-432 administration. This case of T-cell lymphoma suggested the effectiveness of the combined therapy with irradiation and immunopotenciator.
  • —in vitro culture techniqueによる造血障害の検討—
    生田 孝一郎, 佐々木 秀樹, 小磯 良孝, 川島 敏嗣, 大西 三郎, 松山 秀介, 溝口 秀昭, 三浦 恭定, 高久 史麿
    1980 年 21 巻 12 号 p. 1898-1903
    発行日: 1980年
    公開日: 2009/01/26
    ジャーナル 認証あり
    A ten-month-old boy with Shwachman's syndrome (chronic neutropenia and exocrine pancreatic insufficiency) was evaluated.
    Hemopoietic disorder in this case was studied by in vitro culture technique. An assay of bone marrow granulocyte-macrophage precursor (CFU-C) in a double agar culture system demonstrated its reduced number. Production of colony stimulating factor (CSF) from patient's peripheral blood leukocytes appeared normal when tested on normal marrow. No serum inhibitor against CFU-C could be demonstrated.
    The reduced number of erythroid progenitor cells (CFU-E) was shown in a plasma clot culture system.
    These findings suggested that the bone marrow dysfunction in this case might be resulted from a stem cell disorder.
  • 御供 泰治, 樋上 昌男, 仁田 正和, 吉川 公章, 平野 真, 細井 惇, 川村 秀和, 山本 正彦, 立松 正衛, 伊東 信行
    1980 年 21 巻 12 号 p. 1904-1908
    発行日: 1980年
    公開日: 2009/01/26
    ジャーナル 認証あり
    A 33-year-old man was admitted because of abdominal distension on May 23, 1975. A diagnosis of chronic myelocytic leukemia (CML) was made because of leukocytosis, splenomegaly, Piladelphia chromosome (Ph1) positive and a low score of neutrophil alkaline phosphatase activity (NAP). The splenectomy was performed on the chronic phase after 2 years 4 months from the diagnosis in order to prevent the devolopment of acute crisis in a spleen. In this case, no agent has been administered after the splenectomy except a small amount of vercyte immediately after the operation. Lymphoadenopathy, disseminated intravascular coagulation (DIC) and leukemic meningitis were developed at the final course. The variation of leukocyte and platelet count were observed, and the leukocyte count increased to 228×103/cmm and then showed a plateau before the acute crisis. Cell infiltration was recognized rather the generalized lymphnodes than the liver by the pathological findings, and cell kinetics after a splenectomy of CML was discussed. A new protocol of chemotherapy after the splenectomy of CML to induce a more prolonged survival will be expected, and then an effect of the splenectomy should be discussed.
  • 高野 康雄, 知念 俊明, 小川 一誠, 加藤 洋, 北川 知行
    1980 年 21 巻 12 号 p. 1909-1915
    発行日: 1980年
    公開日: 2009/01/26
    ジャーナル 認証あり
    A 53 years old mastectomized woman for breast cancer treated with radiotherapy (total doses 12,600 rad) and with long term oral administration of cyclophosphamide (CPM) and ftorafur (FT), developed aplastic anemia and thereafter acute myelogenous leukemia. About six months after discontinuation of the above therapies, she developed anemia and leukopenia and was referred to our clinic.
    Hematological improvement was obtained by the administration of anabolic hormone, however, two months later she became pancytopenic again. At that time, quite atypical myeloblasts contained peroxidase positive granules, were found 39% in the peripheral blood and 89.4% in the bone marrow, respectively. Leukemic hiatus was present. A bone marrow biopsy revealed coexistence of leukemic cells and breast cancer cells. A diagnosis of breast cancer complicated with acute myelogenous leukemia was made. A combined therapy of adriamycin, CPM and FT was ineffective. OAP regimen of vincristine, cytosine arabinoside and predonisolone revealed transient hematologic improvement. Finally, the patient died of septicemia due to klebsiella. Autopsy revealed wide spread coexistence of leukemia and cancer in the bone marrow, liver, and thyroid.
    The authors discuss some possible explanations for development of acute leukemia after radiotherapy and chemotherapy.
  • Antibody-Dependent Cell-Mediated Cytotoxicity (ADCC)と溶血機序
    高屋 正敏, 市川 幸延, 有森 茂
    1980 年 21 巻 12 号 p. 1916-1922
    発行日: 1980年
    公開日: 2009/01/26
    ジャーナル 認証あり
    A 21-year-old male patient was admitted because of acute onset of generalized purpura and thrombocytopenia (0.2×104/cmm) in Dec., 1977, and treated with prednisolone (PSL) under the diagnosis of idiopathic thrombocytopenic purpura. In Sept., 1979, he noticed jaundice and general malaise. Marked anemia and mild splenomegaly were demonstrated. Examinations of peripheral blood showed normocytic anemia (Hb 5.2 g/dl), leukocytosis (10,700/cmm), thrombocytopenia (6.1×104/cmm), and reticnlocytosis (12.2%). Hemolytic anemia was confirmed by other laboratory data, such as indirect bilirubin, serum LDH, serum iron, and myelogram. Both direct and indirect antiglobulin test were positive, and anti-erythrocyte antibody in his serum was confirmed as IgG. Treatment with large dosage of PSL (100 mg/day) was started. Anemia and then thrombocytopenia were gradually improved.
    Antibody-dependent cell-mediated cytotoxicity (ADCC) of human peripheral leukocytes (PBL) was investigated by using 51Cr-labelled human erythrocytes sensitized with the patients serum. It was concluded that ADCC of PBL in this case was not participated in the mechanism of hemolysis, because neither cytotoxic nor phagocytic activity of PBL was demonstrated against target erythrocytes.
  • 谷脇 雅史, 前川 平, 枝川 潤一, 薗田 精昭, 井出 透, 沢井 公和, 三沢 信一, 阿部 達生, 瀧野 辰郎, 沢田 博義
    1980 年 21 巻 12 号 p. 1923-1930
    発行日: 1980年
    公開日: 2009/01/26
    ジャーナル 認証あり
    TdT positive and subsequently occurred TdT negative blast crises were observed in a 49-year-old patient with Ph1-positive CML. In the first crisis, blast cells of the patient showed lymphoid morphology (L1) and they had high TdT activity and surface markers of non-T cell, such as negative surface immunoglobulin, positive C3-receptor, Fc-receptor and Ia-like antigen. In the second crisis, however, the blast cells were considered myeloblasts (M1) based on morphologic impression and showed no TdT activity. Both crises responded well to VP therapy. This case suggests that loss of TdT activity in blast cells does not always imply the emergence of cells resistant to VP therapy. The relationships between TdT, VP therapy and karyotype and the origin of target cell in the blast crisis were discussed.
  • 上瀬 英彦, 麦島 秀雄, 三間屋 純一
    1980 年 21 巻 12 号 p. 1931-1937
    発行日: 1980年
    公開日: 2009/01/26
    ジャーナル 認証あり
    5-year-old-girl with recurrent subcutaneous bleedlings and epistaxis was admitted to Shizuoka Children's hospital for further investigation. She had a history of abnormal post-traumatic bleeding of the tongue at the age of 3 years. Hemostatic screening tests on admission revealed prolonged A-PTT and abnormal TEG patterns. She had low level of VIII: C (7.6%∼16%) but normal level of VIII R: AG (115%) and VIII R: WF (94%) Platelet count, bleeding time, PT, fibrinogen, Platelet retention, ristocetin induced platelet aggregation and factor VIII-crossed immunoelectrophoresis showed normal values. No factor VIII inhibitor was demonstrated. Her Karyotype revealed 46 chromosomes with two X chromosomes. Mother showed no abnormal hemostatic figures, but father was bleeder, althrough he was not precisely tested. From these findings, she was diagnosed to be a female hemophilia A of heterozygous type.
  • 丸尾 國造, 吉川 治哉
    1980 年 21 巻 12 号 p. 1938-1943
    発行日: 1980年
    公開日: 2009/01/26
    ジャーナル 認証あり
    The 33-year-old-man was admitted because of purpura and pain above the both legs. One month ago, he suffered from lacunar tonsillitis and was treated with antibiotics.
    On admission, he appeared actually ill, with petechiae and purpuric spots over the extremities and the face. He complained of severe pain in the both thighs, though neither muscle weakness nor atrophy was prominent. The serum creatinine phophokinase value was 41 units (normal: 0 to 35 U) and 24-hour excreations of creatine in urine was 572 mg. Test for C-reactive protein was positive; antistreptolysis 0 titer (ASLO) was 480 Todd units; antistreptokinase titer (ASK) was 81,920 U; Electromyogram revealed a pattern of low amplitude neuromuscular units.
    Clinical signs and symptoms suggested his disease as vascular purpura with polymyositis syndrome. Microscopical findings of the muscle showed to be uneven muscle fibers and wide-opened endomysim. They were compatible with polymyositis of vasculo-interstitial type.
    Five months later, he suffered from glomerulonephritis.
    Considering that the common pathognomonic factor between the vascular purpura and polymyositis is an angitis, it could be presumed that the infection with hemolytic streptococcus caused the angitis as well as glomerulonephritis.
  • 奈良 信雄, 宮本 忠昭, 栗栖 明, 恒元 博, 遠藤 一靖, 浦井 利雄, 和田 全玄
    1980 年 21 巻 12 号 p. 1944-1950
    発行日: 1980年
    公開日: 2009/01/26
    ジャーナル 認証あり
    Two siblings with Fanconi's anemia developing squamous cell carcinomas were reported.
    Case 1, a 23-year-old male, was diagnosed as Fanconi's anemia in November, 1975. He was in a small stature with small head, hyperpigmentation, severe pancytopenia and chromosomal abnormalities (chromatid breaks, exchanges, gaps, etc.). He had been treated with methenolone acetate until June, 1979, when carcinoma of the right lower gum was noticed. With irradiation therapy by electron and fast neutron beams, tumor regressed gradually. He was well under follow-up in April, 1980 at present.
    Case 2, a 14-year-old female and an elder sister of the patient in case 1, was diagnosed as aplastic anemia in July, 1969. Treated with prednisolone followed by methenolone acetate, she died of gingival and esophageal carcinomas 7 years later.
    Consanguinity of their parents was noticed. The occurrence of Fanconi's anemia associated with squamous cell carcinomas in two siblings suggested genetic factor as an etiology of the disorders. The pathogenesis of congenital aplastic anemia and oncogenesis were discussed.
  • 近藤 邦夫, 石野 千津子, 大竹 茂樹, 塩原 信太郎, 末永 孝生, 森 孝夫, 幸道 秀樹, 吉田 喬, 舟田 久, 中村 忍, 服部 ...
    1980 年 21 巻 12 号 p. 1951-1958
    発行日: 1980年
    公開日: 2009/01/26
    ジャーナル 認証あり
    Autologous bone marrow transplantation (Auto-BMT) was carried out a 27 year old man with stage IV malignant lymphoma of 4 months' duration. This procedure was performed under bioclean room therapy, after high dose cyclophosphamide (CY), 120 mg/kg and 1,000 rad total-body irradiation (TBI). The autologous marrow was removed 2 months before BMT, gradually frozen in the anticryopreservation and kept in liquid nitrogen until use. Just before use, the cryopreserved marrow was thawed rapidly, 2 times washed and intravenously infused to the patient. Sequential blood study showed WBC of 0 on day +5, but WBC of over 1,000 on day +21. He has survived from gangrenous stomatitis and acute hepatitis by intensive supportitive therapy such as hyperalimentation, fractionated blood transfusion, intravenous immunoglobulin, etc. He is now healthy with no chemotherapy, approximately 6 months after BMU. This is the first successful cases in Japan of Autp-BMT aimed to cure the patient. In summation, to malignant tumors with no marrow invasion by tumor cells and with hypersensitivity to drugs or TBI, Auto-BMT would be the most potential therapeutic weapon.
  • 木須 達郎, 渋谷 恒文, 自見 庄三郎, 仁保 喜之, 松隈 敬太, 菊池 昌弘
    1980 年 21 巻 12 号 p. 1959-1965
    発行日: 1980年
    公開日: 2009/01/26
    ジャーナル 認証あり
    A case of myeloproliferative syndrome manifested by fever, hepatosplenomegaly and pancytopenia was described. This 21 year-old Japanese female patient had a outcome of fatal course after nine months in spite of various treatments. The diagnosis of this case was very difficult although many investigations were done. We had suspected malignant lymphoma or malignant reticulosis, but could not diagnose definitely before autopsy.
    There was marked hepatosplenomegaly, but no evidence of the swelling of lymph nodes were seen. The autopsy showed neither tumor formation nor focus of infection, and bone marrow revealed a compact red marrow. The microscopic examination revealed the marked increase of erythroblastic, granulocytic and megakaryocytic series in the bone marrow, and marked infiltration of mature neutrophils and erythroblasts in the sinusoid of the liver. This was also seen in the spleen and lymph nodes.
    These findings were compatible to the myeloproliferative syndrome advocated by Dameshek in 1951. This syndrome includes acute and chronic granulocytic leukemia, polycythemia vera, acute and chronic myelosclerosis with myeloid metaplasia, Di Guglielmo syndrome, thrombocythemia and other disorders of the proliferation of one or more marrow constituents. In this context, this rare case was included into myeloproliferative syndrome but did not belong to any single entity of this syndrome.
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