臨床血液
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
22 巻, 11 号
選択された号の論文の18件中1~18を表示しています
綜説
臨床研究
  • 新倉 春男, 萩原 昭二, 西山 博明, 森 啓, 毛利 博, 松野 一彦, 寺田 秀夫
    1981 年 22 巻 11 号 p. 1700-1707
    発行日: 1981年
    公開日: 2009/01/26
    ジャーナル 認証あり
    Twenty two patients with polycythemia vera (PV) and 11 patients with essential thrombocythemia (EF) were treated with an alkylating agent, carboquone.
    No patients received chemotherapeutic regimen prior to carboquone therapy. Carboquone was administered orally in 0.5∼2.0mg daily dose as remission induction therapy without simultaneous administration of other chemotherapeutic agents. Most patients were administered smaller doses of carboquone (0.25∼1.0mg/day) as a maintenance therapy. Phlebotomy was combined with carboquone therapy in 16 patients with PV during remission induction.
    All patients responded to carboquone therapy. Complete remission was obtained in 21 out of 22 patients with PV and in 10 out of 11 patients with ET. IT required median 41 days and 37 days to achieve remission in patients with PV and with ET, respectively. The median duration of remission without carboquone was 126 days and 76 days in PV and in ET, respectively.
    No serious side effect was noted. Only one patient with ET complained of nausea and vomiting, because of which carboquone was discontinued. Two patients developed bone marrow suppression (pancytopenia) which was promptly recovered by discontinuation of carboquone.
    These data indicate that carboquone is markedly effective for the treatment of PV and ET.
  • —主にアポトランスフェリンの効果—
    川上 義, 曽根 良治, 沼尻 志信, 坂田 堯
    1981 年 22 巻 11 号 p. 1708-1713
    発行日: 1981年
    公開日: 2009/01/26
    ジャーナル 認証あり
    Congenital atransferrinemia is a rare disease which exhibits an extreme hypochromic anemia due to transferrin deficiency. We have been following this case for about 17 years but we have experienced considerable difficulties with the side effects of repeated blood and plasma preparations transfusion. However, in October of 1977 we obtained continuous use of this drug, we have achieved a remarkable improvement of anemia. At first, we administered a dosage of 300mg/day. Then we decreased the dosage and we now are administering 1,500mg/week. Although the level of serum transferrin was low despite this therapy, it produced a sufficient effect on anemia. No side effects due to this therapy have been observed. Although Apotransferrin therapy is not yet established, we thought it useful to report on our clinical experinece with this agent in regard to the dosage and its effectiveness in treating congenital atransferrinemia.
  • —特にReceptor濃度と治療成績について—
    清水 信, 浅尾 豊彦, 井戸 正流, 神谷 斎, 桜井 実
    1981 年 22 巻 11 号 p. 1714-1719
    発行日: 1981年
    公開日: 2009/01/26
    ジャーナル 認証あり
    Glucocorticoid receptor level in acute leukemic blast cells in childhood was examined. The receptor level was quite variable in leukemic cells among patients with acute lymphoblastic leukemia (ALL), but the mean receptor level was distinctly higher as compared with that of acute myeloblastic leukemia. In ALL, there was an apparent correlation between prognosis of leukemia and glucocorticoid receptor level. When leukemia (ALL) relapsed, glucocorticoid receptor level of blast cells was lower than that of pretreatment leukemia.
  • 大竹 茂樹, 山村 真由美, 尾高 和亮, 手島 博文, 塩原 信太郎, 小林 三郎, 末永 孝生, 石野 千津子, 吉田 喬, 舟田 久, ...
    1981 年 22 巻 11 号 p. 1720-1728
    発行日: 1981年
    公開日: 2009/01/26
    ジャーナル 認証あり
    To evaluate the safety and usefullness of central venous access (CVA) and intravenous hyperalimentation (IVH) for the efficient management of patients with hematological malignancy, 33 central venous catheters were inserted into 26 patients with hematological disease. A median count of platelets on the day of insertion was 5.3×104/cmm (range: 0.8-25.4×104), and in two cases exit site bleeding was experienced but easily stopped by pressure. The catheters were in place for a median of 26 days (range 3-78), and used for not only the administration of fluids, blood components and intravenous drugs, but also collecting blood samples.
    As far as side effects of CVA are concerned, no exit site infection was seen in spite of severe granulocytopenic state and frequency of septicemia (15.2%) seen in patients with CVA was of no significant differece from that in control patients not receiving CVA. In the cases receiving IVH, nutritional parameters remained stable in spite of poor oral intake.
    These observations suggest that CVA and IVH facilitate patient care, minmizing discomforts of patients and thus, at times elongate patient's survival.
症例
  • 中島 一格, 柴田 皓示, 平井 浩一, 茂木 安平, 吉村 隆, 田所 憲治, 鈴木 修二, 村中 正治
    1981 年 22 巻 11 号 p. 1729-1736
    発行日: 1981年
    公開日: 2009/01/26
    ジャーナル 認証あり
    A case of cold agglutinin disease associated with malignant lymphoma was reported. The patient was 69-year-old male who was referred to our clinic because of fever, easy fatigability and Raynaud's phenomenon. On admission, anemia (Hb 6.9g/dl), splenomegaly, generalized lymphadenopathy and erythroid marrow (NCC 342,500/μl, ME ratio 0.35) with no abnormality of myeloid and lymphoid series were found. The serum haptoglobin and complement (CH50) were 8.8mg/dl and 11.4, respectively. The direct Coombs test was positive and serum IgM was 382mg/dl. The serum cold agglutinin showed very high titer (1: 65, 536). Eluted cold agglutinin belonged to monoclonal IgM globulin. It had λ-light chains. The agglutinin titer was remarkably higher against adult O RBC than against cord red cells, which meant its anti-I-specificity. Biopsy of a left supraclavicular lymphnode revealed diffuse lymphosarcoma, lymphoblastic (WHO). The patient was treated with combination of vincirstine, cyclophosphamide and prednisolone. Anemia, lymphadenopathy and splenomegaly improved. About one year after the first admission, the patient died because of aggravated hemolytic anemia, liver damage and leukemic conversion of malignant lymphoma.
  • 津本 清次, 大薮 博, 末吉 公三, 陰山 克
    1981 年 22 巻 11 号 p. 1737-1742
    発行日: 1981年
    公開日: 2009/01/26
    ジャーナル 認証あり
    A 26-year-old pregnant housewife at the 22nd week of gestation was admitted to the hospital on April 9, 1979 with the presenting symptoms of bleeding tendency and edema. Hematologic findings on admission were: Hb 8.0g/dl; RBC 243×104; platelet count 8,000; WBC 10,600; a predominace of leukemic cells, which accounted for 48% of the total white cell count. Bone marrow study showed that the nucleated cell count was 140,000 (64.7% of which were accounted for by leukemic cells) and there was hypoplasia of the erythroblastic and megakaryocytic series.
    In view of the presence of symptoms of heart failure and bleeding tendency, platelet-rich plasma and steroids were given, along with transfusion of fresh blood, as an initial regimen and then therapeutic abortion was done. Subsequently the patient was placed on a combination antileukemia chemotherapeutic regimen of DCMP. After this therapy brought about remission, a remission maintenance therapy with Ara-C, 60-80mg once a week intravenously, and MTX, 10mg twice a week orally, was instituted together with an adjunctive immunotherapy with OK-432. Remission enhancement therapy has been given at 4-week intervals and the patient has been kept in a state of complete remission.
    A case of leukemia complicating pregnancy was reported with some comments on therapeutic concept of the malignancy in those particular clinical circumstances and a review of pertinent literature.
  • —再不貧様前白血病状態との関連を中心に—
    西尾 晃, 中西 忍, 北村 誠, 芦沢 博, 浜見 拓哉, 中田 哲夫, 細田 光蔵, 春山 春枝, 香月 昭人, 杉島 聖章, 伊地知 ...
    1981 年 22 巻 11 号 p. 1743-1750
    発行日: 1981年
    公開日: 2009/01/26
    ジャーナル 認証あり
    The peripheral blood picture of this patient was pancytopenia without any leukemic cells, but the bone marrow cellularity was markedly hyperplastic in both aspiration films and biopsied specimen with massive leukemic cell infiltration at the initial stage. The clinical course of this case was thereafter so-called “smoldering”, and administered no anti-leukemic agent and OK-432 alone. The peripheral blood picture was stationary for 17 months, meanwhile bone marrow hypercellularity had gradually and constantly decreased and the leukemic cell percentage had selectively regressed. In this stage, it became difficult to diagnose acute myelogenous leukemia. At autopsy, no leukemic cell infiltration was discovered in any organs without bone marrow.
    The hypoplastic acute leukemia in aged patients demonstrates smoldering clinical course and bone marrow aplasia. The hypocellularity of bone marrow is not demonstrated at early stage of acute leukemia which will progress to overt typical lekemia but the terminal feature of the overt leukemia in bone marrow. The pathogenesis of this peculiar leukemia has been analized in the proliferating ability of leukemic cells, in the immunological defense mechanism of host and in the biological state of aging, but it has not been determined that hypoplastic acute leukemia is one of the entity of acute myelogenous leukemia.
    Accompaning with the increasing incidence of acute leukemia in aged patients, hypoplastic acute leukemia is not to be rare, and the conservative treatment may be possible to promise relatively long survival, and radical chemotherapy will must be necessary, and the establishment of the criteria for the radical treatment is seriously recommended.
  • —特にAIHA·PRCAとAILDの病因的相関について—
    萬納寺 正清, 下田 雅大, 是沢 俊輔, 山田 治, 戸川 敦, 八幡 義人, 梅村 創, 小鶴 三男
    1981 年 22 巻 11 号 p. 1751-1758
    発行日: 1981年
    公開日: 2009/01/26
    ジャーナル 認証あり
    In this report, we present a case of angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) associated with autoimmune hemolytic anemia (AIHA) and pure red cell aplasia (PRCA).
    A 71 year-old man was admitted to our hospital with fever, severe anemia and rapidly enlarging generalized lymphadenopathy. A diagnosis of AILD was confirmed by lymph node biopsy. Coombs test was positive and cold agglutinin titers were substantially elevated (×8192). Hemoglobin and hematocrit were markedly decreased (Hb: 4.9g/dl, Ht: 13.8%) with no reticulocyte in the peripheral blood and no erythroid precursor cells in the bone marrow. Bone marrow culture studies showed (1) decreased numbers of erythrocytic colony forming units (CFU-E) (19.8 colonies/105 cells: control 72.9) and (2) suppression of normal human CFU-E by IgG in the patient's serum (suppression rate: whole serum 40.1%, IgG 29.8%). It suggests that the positive Coombs test, the elevation of cold agglutinin titer and the presence of the IgG inhibitor erythropoiesis could be attributed to AILD, which developed AIHA and PRCA.
    After 12 weeks of treatment with prednisolone (60mg/day), vincristine (1.0mg/wk), azathioprine (50mg/day) and anabolic steroid (400mg/wk), AILD was improved at first and then AIHA and PRCA, sequentially. After 3 months of the treatment, the patient developed subacute hepatitis and died from hepatic failure and pneumonia.
    At autopsy, there was complete recovery from AILD and erythroid precursor cells were seen normally in the bone marrow. In addition, pneumocystis carinii bronchopneumonia and dissemination of aspergillosis were found.
    A case of AILD with AIHA and PRCA has been presented with discussion of its possible pathogenesis.
  • 幸道 直樹, 西村 理, 新川 正治, 今宿 晋作, 楠 智一
    1981 年 22 巻 11 号 p. 1759-1765
    発行日: 1981年
    公開日: 2009/01/26
    ジャーナル 認証あり
    A six month old male infant was admitted because of pallor, large head and multiple skin nodules at the frontoparietal region. Hemoglobin concentration was 7.3 g/dl, white blood count 17,200/cmm with 42% malignant cells. Bone marrow smear had malignant cells, accounting for 59% of the total. Spinal fluid cytology revealed an infiltration of tumor cells into the leptomeninges. In addition, brain CT scan revealed the existence of bilateral subdural effusion, which was also infiltrated with malignant cells. We also noted that the mitotic index of the cells in the subdural effusion was higher than that in the CSF and bone marrow. Biopsied specimen of the skin nodules was identified to be a lymphoma or leukemia cutis. Subsequently, the patient was diagnosed as leukemia/lymphoma due to the difficulty in differentiating these two malignancies.
    Complete remission was achieved after one month of chemothrapy, and drainage surgery was performed to remove subdural fluids. However, the patient relapsed and finally died at age of 11 months. Autopsy revealed an infiltration of malignant cells into the dura matter, leptomeninges and other organs.
    A very uncommon assocation of leptomeningeal leukemia and leukemic subdural effusion is discussed.
  • —症例報告と文献的考察—
    菱谷 好高, 加納 正, 茂在 敏司, 貴島 康彦
    1981 年 22 巻 11 号 p. 1766-1772
    発行日: 1981年
    公開日: 2009/01/26
    ジャーナル 認証あり
    The simultaneous occurrence of multiple myeloma and atypical leukemia was reported in a patient who had not undergone any chemotheray.
    A 76-year old man, in whom anemia had been noted since 20 months earlier, was hospitalized in October, 1979, because of fatigability and high fever. Physical examination revealed a severe pallor without swelling of lymphnode, liver or spleen. Laboratory tests revealed the following: hemoglobin was 2.7 g/dl, hematocrit 7.9%, RBC 75×104/mm3, WBC 3,200/mm3 with segmented neutrophils 55%, eosinophils 3%, monocytes 2%, lymphocytes 36%, myeloblast 1%, promyelocytes 2% and plasma cell 1%, and electrophoresis revealed M-component (1.9 g/dl) in β area which was identified as IgA of λ type by immunoelectrophoresis. The IgG and IgM levels in the serum were reduced and Bence Jones protein (λ type) was demonstrated in the urine. Bone marrow aspiration revealed a normocellular marrow with 10.8% of myelobasts, 14.4% of promyelocytes and 9.2% of myeloma cells which produced IgA of λ type exclusively.
    It is well known that acute leukemia may develop in patients with multiple myeloma. Most of these patients had undergone long term chemotherapy with alkylating agents such as melphalan. The development of acute leukemia in these cases has been attributed to the therapy.
    The patient reported here is a very rare case which shows simultaneous occurrence of multiple myeloma and acute leukemia without prior chemotherapy or irradiation.
    Accumulation of such cases is desirable to ellucidate the relationship between multiple myeloma and acute leukemia.
  • 福田 光之, 福島 幸隆, 三浦 亮, 前多 治雄, 千田 勝一, 藤原 哲郎, 樋口 誠一
    1981 年 22 巻 11 号 p. 1773-1780
    発行日: 1981年
    公開日: 2009/01/26
    ジャーナル 認証あり
    A 24 year old woman, who was diagnosed as acute myeloid leukemia late in the first trimester, received an intense antileukemic combination chemotherapy during the pregnancy.
    A premature female infant was delivered at 32 weeks gestation. Physical examination of the neonate at birth revealed no significant abnormalities, but there could be found about 10×104/cmm of nucleated erythrocytes and a few lymphoid mononuclear cells but no neutrophiles could be found in the peripheral blood. Aspirated bone marrow was markedly hypocellular. Jaundice developed rapidly, and exchange blood transfusion was performed two times, 400 ml in total, and further clinical course became favorable with gradual improvement of peripheral blood picture. Bone marrow aspirated on 20th day remained still hypocellular but its picture showed no significant abnormalities.
    Mother died of aggravation of leukemia two months after delivery but her child, now 3 year old, enjoys good health.
    The blood dyscrasia of this child was thought to be the influence of antileukemic agents given to her mother during the pregnancy.
  • —本邦での腫瘤形成性慢性骨髄性白血病の文献的考察—
    斎藤 嘉美, 野中 泰延, 行徳 祐一
    1981 年 22 巻 11 号 p. 1781-1787
    発行日: 1981年
    公開日: 2009/01/26
    ジャーナル 認証あり
    Most cases with chronic myelogenous leukemia (CML) usually terminate in blastic crisis. But, there are some cases with CML, in whom tumor-formation has appeared without or before blastic crisis.
    We presented an autopsied case of CML, who showed a painful tumor formation at the left knee joint with atypical blastic crisis. A 31-year-old housewife admitted to our department of Tokyo University on Jan. 13, 1975, with a chief complaint of visual disturbance and splenomegaly. She was diagnosed as CML from the peripheral blood and bone marrow findings, a low value of NAP score and rate, and positive Ph1. During four years, administration of busulfan has brought about clinical improvement and good control.
    On Feb. 1, 1979, she complained of severe pain in the left knee without roentgenographic abnormality. On Feb. 26, local tenderness and swelling was disclosed, and hematological finding of peripheral blood (myeloblast 9.5%, promyelocyte 19.0%), increasing splenomegaly and refractory leukocytosis were suggestive of atypical blastic crisis. Immediately combination therapy (VP and VEP) were tried, and brought about good result.
    On March 20, the similar in the right knee, where tumor formation in the soft tissue was disclosed 6 weeks later, and thereafter an osteolytic lesion of right femur was revealed by roentgenography and CT scanning. Local irradiation of X-ray with total dosis of 2500 rad. and intravenous administration of cytosine arabinoside with total dosis of 800 mg for a period 10 days, brought about clinical improvement with diminished size of lesion.
    One month later, multiple painful tumors developed at the knee, elbow and shoulder, followed by hematological blastic crisis. Though intensive combination therapy (DCP) was instituted, no effect was obtained, and on July 26, 1979, she was died with bronchopneumonia.
    Pathological diagnosis in autopsy was CML with blastic crisis and tumor formation of the right knee joint, gum and right kidney.
    Eighty-two cases of CML with tumor formation reported in Japanese literature were analysed.
  • —腫瘍細胞のin vitro parathyroid hormone productionに関して—
    安田 佳織, 名取 英世, 江上 康一郎, 児玉 研一, 山口 達夫, 加地 正郎
    1981 年 22 巻 11 号 p. 1788-1793
    発行日: 1981年
    公開日: 2009/01/26
    ジャーナル 認証あり
    A 42-year old man with jaundice, general fatigue, nausea and weight loss was admitted to our hospital. White blood cell (WBC) count was 40,400/cmm and 60 per cent of the peripheral WBC and 35.2 per cent of bone marrow cells were lymphoid mononuclear cells. Some of them possessed convoluted and lobulated nuclei. In the examination of peripheral lymphocytic subpopulation, E rossette forming cells were 86.0 per cent and EAC rossette forming cells were 0 per cent. The diagnosis of Adult T-cell leukemia was made.
    Abnormal blood findings inculuded a serum calcium of 15.1 mg per deciliter and elevated immunoreactive parathyroid hormone. In order to demonstrate the production of parathyroid hormone from the leukemic cells, peripheral lymphocytes were cultured. A patient with normocalcemic acute lymphocytic leukemia and a healthy man were studied in identical fashion as control. Immunoreactive parathyroid hormone level in cultured medium from the present case was higher than that from other two controls.
  • 野尻 外士雄, 白幡 聡
    1981 年 22 巻 11 号 p. 1794-1800
    発行日: 1981年
    公開日: 2009/01/26
    ジャーナル 認証あり
    A case of hemolytic uremic syndrome is reported. The symptoms of this three-year old boy were severe abdominal pain and frequent bloody diarrhea on admission, and later thrombocytopenia, hemolytic anemia, uremia (triad of HUS), fever, and disturbances of consciousness developed.
    Coagulation studies of the patient's plasma were carried out before manifestation of HUS, and continued during the acute phase. Plasminogen and α2-plasmin inhibitor were decreased remarkably during the prodromal phase, and recovered gradually to normal range in the early acute phase. Serum FDP had already been increased before thrombocytopenia appeared and increased further during the acute phase. Platelet aggregation rate during the prodromal phase was slightly reduced.
    A clear rise was seen in antibody titer against adeno-virus, and the titer of serum circulating immune complex was very high. It has been suggested that immune complexes produced by some infection could have activated fibrinolytic and/or complement system and had platelet aggregating activity.
    By administration of flurbiprofen and dipyridamole, the platelet count increased proptly.
  • 岩田 展明, 徳江 泰, 東雲 正剛, 宮脇 修一, 宮尾 誠一, 小峰 光博, 土屋 純, 前川 正
    1981 年 22 巻 11 号 p. 1801-1808
    発行日: 1981年
    公開日: 2009/01/26
    ジャーナル 認証あり
    A seven-year-old boy was admitted because of excessive bleeding following tooth extraction. Anemia and petechial hemorrhage were noted together with the palpable spleen. He was pancytopenic with 9.1% reticulocyte. Monocyte and moncytoid cells represented 36% of his peripheral leukocytes. Cytochemical findings supported the myeloid lineage of these cells. Neutrophil alkaline phosphatase score was not reduced, and serum muramidase was elevated to 25 μg/ml. The monocyte and monocytoid cells accounted for 18% of the normocellular marrow aspirate. Dysmyelopoietic features were evident in all three hematopoietic cell lines. Karyotype analysis indicated the establishment of an abnormal clone in the patient's bone marrow (50XY, +i (lq), +8, +marker 1, +marker 2), whereas the PHA-stimulated peripheral leukocytes showed 46XY. Hemoglobin analysis revealed Hb F; 72% and Gγ/Aγ 7/3. Expression of I antigen was significantly reduced in the patient's red cells. However, the most striking finding was that the carbonic anhydrase activity of the patient's red cells did not differ from that of normal adult's red cells. Carbonic anhydrase B was detected normally on the cellulose acetate membrane electrophoresis of the patient's hemolysate. Zinc content was also not reduced. Such a remarkable dissociation of the increased Hb F from unreduced carbonic anhydrase activity has not, presumably, been reported thus far in the juvenile CML. He died of sepsis 17 months after the initial presentation of the disease.
  • 北 堅吉, 巽 英二, 那須 芳, 滝内 鳩子, 白川 茂, 高月 清, 内野 治人, 永尾 正男, 田中 暁, 伊藤 忠弘
    1981 年 22 巻 11 号 p. 1809-1814
    発行日: 1981年
    公開日: 2009/01/26
    ジャーナル 認証あり
    A case of T-cell lymphoma associated with M-component in terminal stage is reported. A 67-year-old woman suffering from mitral stenosis was admitted to Shizuoka city hospital because of dyspnea on exertion and right cervical lymphadenopathy. Biopsy of lymphnode showed Hodgkin-like histological features with diffuse destruction of lymphfollicles. The neoplastic cells revealed marked pleomorphism and formed E-rosettes. Negative Mantoux reaction and high titer of anti-early antibody of EB virus were also noted.
    She was treated with VEMP therapy, which was stopped because of cardiac failure. About 2 months later serum M-component (IgG kappa) appeared rapidly without Bence Jones proteinuria, and she died of cerebral thrombosis. Autopsy showed the wide infiltration of neoplastic cells into many organs.
    Out of 18 cases of nonleukemic T-cell lymphoma examined in our laboratory, 7 cases showed mild or moderate polyclonal gammopathy, but the patient with M-component was just only this case. It suggested that M-component might appear due to inappropriate regulation of immune system in the presence of T-cell lymphoma.
例会
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