巻号一覧
23 巻, 11 号
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臨床研究
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小林 直, 小川 一誠, 稲垣 治郎, 堀越 昇, 江崎 幸治, 井上 雄弘, 相羽 恵介, 永田 隆樹, 知念 俊昭, 倉石 安庸, 目黒 ...1982 年 23 巻 11 号 p. 1681-1688
発行日: 1982年
公開日: 2009/01/26
ジャーナル 認証ありTwenty five patients with acute non-lymphocytic leukemia were treated with DCTP (I) regimen consisting of daunomycin 40 mg/m2 IV on day 1, cytosine arabinoside 40 mg/m2 diluted with 500 ml of 5% glucose by drip infusion twice a day for 5 days, 6-thioguanine 60 mg/m2/day orally for 10 days and prednisolone 60 mg/m2/day orally for 5 days. This regimen was repeated in every 2∼3 weeks intervals untill obtaining complete remission and maintenance therapy was principally the repetition of the same regimen in every one or two months.
The complete remission rate was 79% in 24 evaluable patients but 83% in 18 previously untreated patients. The median duration of remission was 28 weeks and median survival time was 56 weeks in responders, whereas it was 6 weeks in non-responders.
Hematologic toxicity was a dose limiting factor but this was clinically manageable. However hepatotoxicity was occured in 64% of patients but in the majority of patients this was moderate and reversible, and this toxicity seemed to be relating to blood transfusion in most patients.
DCTP (I) combination chemotherapy is highly effective for the remisson induction of adult ANLL. However further studies in order to improve the duration of remission are necessary in future.抄録全体を表示PDF形式でダウンロード (623K) -
中田 安成, 片岡 幹男, 山下 二郎, 佐藤 俊雄, 野田 憲男, 小林 洋三, 大田原 保幸, 岸 俊行, 林 恭一, 大熨 泰亮, 木 ...1982 年 23 巻 11 号 p. 1689-1695
発行日: 1982年
公開日: 2009/01/26
ジャーナル 認証ありFunctions of peripheral blood monocytes were studied in total of 34 patients with malignant lymphoma, i.e. 17 untreated patients and 17 patients in complete remission, and 50 healthy controls.
The mean absolute numer of monocytes was 241±140/cmm (M±SD) in the untreated malignant lymphoma patients and 299±144/cmm in controls. The difference between these two groups was not significant. The level of beta-galactosidase activity was 34.2±13.9 in patients and 35.4±10.7 in controls, and there was no significant difference between two groups. By contrast, the ability of phagocytosis was significantly lowered in patients (53.1±23.0) compared to healthy controls (82.1±9.5) (p<0.01). The monocyte chemotactic response was also significantly decreased in patients (23.6±5.4) compared with that of controls (34.8±8.0) (p<0.001). Approximately 30% of the patients had depressed the monocyte cytocidal activity against tumor cells with value below the ninety-fifth percentile of the activities of healthy controls.
In the malignant lymphoma patients in complete remission, the mean number of monocytes and the beta-galactosidase activity were in the mormal range, but both the abillity of phagocytosis and chemotaxis were significantly reduced compared with healthy controls, as well as the untreated patients.
The monocyte functions, the clinical stage and the histological type failed to demonstrate any significant correlation among them. Furthermore, the decreased monocyte functions did not correlate with the presence of cutaneous anergy to PPD, PHA and DNCB.
These data support the hypothesis that depressed monocyte function may contribute to the increased susceptibility to infections of patients with malignant lymphoma and it may be an additional factor favoring tumor dissemination in the disease.抄録全体を表示PDF形式でダウンロード (565K) -
—ヘパリン500単位，2時間毎one shot静注法の有用性—藤井 浩, 清水 忠雄, 関 茂樹, 陶山 芳一, 山本 和子1982 年 23 巻 11 号 p. 1696-1705
発行日: 1982年
公開日: 2009/01/26
ジャーナル 認証ありDespite of extensive experience with heparin, neither the optimal therapeutic dose nor the proper ways of heparin therapy for the treatment of disseminated intravascular coagulation (DIC) have been established.
For 73 patients with acute DIC, the bolus intravenous administration of 500 units (A group) or 1,000 units (B group) of heparin every two hours was done with the administration of 5 units of fresh frozen plasma daily. In 37 patients (65%) of A group and 11 patients (69%) of B group, DIC was improved by this way of heparin therapy. In only two patients of B group, the administration of heparin seemed to provoke the bleeding diathesis.
Repeatedly 500 units of heparin every two hours were administered bolus intravenously to two healthy adults. Then, the heparin effect was observed during 30 minutes and the accumulation effect of heparin was not observed.
The bolus intravenous administration of 10 units/kg of heparin was done to ten patients with liver diseases, DIC or ITP, whose concentration of plasma antithrombin-III showed various degree. Then, it was observed that the heparin effect was extensive because of low concentration of anti-coagulant factors such as fibrinogen, though the concentration of anitithrombin-III was very low.
The bolus intravenous administration of 500 units of heparin every two hours in the safe and useful way of heparin therapy for acute DIC.抄録全体を表示PDF形式でダウンロード (692K) -
森山 美昭, 青木 定夫, 漆山 勝, 大西 昌之, 広沢 秀夫, 小山 覚, 花野 政晴, 布施 一郎, 高井 和江, 長山 礼三, 藤原 ...1982 年 23 巻 11 号 p. 1706-1712
発行日: 1982年
公開日: 2009/01/26
ジャーナル 認証ありBetween 1979 and 1982, 60 adult patients with acute leukemia were prospectively classified according to the FAB system and treated on the DVP (daunomycin, vincristine and prednisolone) or the DC(M)VP (DVP+cytosine arabinoside and/or 6 MP-R) induction therapy for acute lymphocytic leukemia or L type and on the DCMP (daunomycin, cytosine arabinoside, 6 MP-R and prednisolone) protocol for acute nonlymphocytic leukemia or M type.
In L type (L1: 1 and L2: 12 cases), 92.3% complete remission rate was induced with the DVP and DC(M)VP regimens, and 9 out of 13 patients with L type are still alive. In M type (M1: 12, M2: 10, M3: 11, M4: 6, M5: 5, and M6: 3 cases), an overall complete remission rate of 66.7% was achieved with the DCMP regimen, indicating that response of M type to therapy appears to be less than that of L type. In subtype, we obtained remissions in 90% of M 2 and 80% of M 5 patients, respectively. Seven out of 10 patients with M 2 are still alive. However, M 1, M 3 and M 4 patients appeared to do less well than M 2 and M 5 patients according to FAB classification. Especially, M6 patients were found to be poorly prognostic (CR rate: 33.3%). These findings suggest that a good correlation between prognosis and FAB classification is seen on our protocols. Therefore, FAB classification is likely to be useful for diagnosis and treatment of acute leukemia.
On the other hand, we observed a complete remission rate of 53.8% in 13 patients aged 60yr or over treated on the same protocol and only one patinet alive, which is poor prognosis as compared with that of 77.8% in patients aged 16∼59yr. This suggests that age is one of adverse prognostic factors for response and survival in acute leukemia.抄録全体を表示PDF形式でダウンロード (511K)
症例
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曾我 謙臣, 酒井 力, 柴田 昭, 本間 慶一, 根本 啓一1982 年 23 巻 11 号 p. 1713-1718
発行日: 1982年
公開日: 2009/01/26
ジャーナル 認証ありAn autopsy case of 45-year-old male with primary myelofibrosis showing immunological abnormalities and multiple subcutaneous tumors caused by extramedullary hematopoiesis was reported. His clinical course was about one year after the diagnosis was made, and died of pulmonary hemorrhage.
On admission, peripheral blood CFU-C and CFU-E was increased, but disappeared within a month, which would suggest a progressing dysfunction of the hematopoietic cell lines.
Autopsy revealed marked fibrosis of bone marrow and multiple foci of myeloid metaplasia intermingled with various drades of fibrosis. Where hematopoiesis is normally found in the embryonic period, meanwhile some organs showed infiltrative features.
Histologically, extramedullary hematopoietic tissues including skin and atrial cardiac muscles were diffusely infiltrated with myeloblasts, erythroblasts and immature megakaryocytes. Especially, invasive and destructive features were found in the cardiac muscles.
Circulating immune complexes value was more than 2,000 AHGeq/ml both on admission and just before death. In the terminal stage of the clinical course, M-protein turned to be positive and which was revealed IgG (κ) type by immunoelectrophoresis. From the above-mentioned results, the relationship of primary myelofibrosis with immunological abnormalities was discussed.抄録全体を表示PDF形式でダウンロード (630K) -
松崎 博充, 山口 一成, 原 英記, 満屋 裕明, 河野 文夫, 荒木 弘一, 田中 龍二, 岸本 進1982 年 23 巻 11 号 p. 1719-1726
発行日: 1982年
公開日: 2009/01/26
ジャーナル 認証ありA 70-year-old female with the simultaneous occurrence of multiple myeloma and acute myelogenous leukmia without previous chemotherapy was presented. Indirect immunofluorescence and protein A-coupled ox red blood cells (ORBC) rosette technique by use of anti-idiotype (Id) antibody showed that some T cells from the patient had receptors with idiotypic specificity identical with that of the secreted myeloma protein. Plaque forming cell (PFC) assay showed the presence of Id producing peripheral blood lymphocytes with EB virus receptor, probably B cells in the patient. These observations strongly suggested that multiple myeloma was not the result of a neoplastic transformation of the most differentiated B cells, plasma cells, but of lymphoid stem cells that were capable of differentiating into either B or T cells. However, there was not a detectable population of myeloblasts that expressed the same idiotype. Chromosomal analysis revealed a deletion of the long arm of chromosome 8 in the myeloblasts but not in T or B cells from the patient. These results convinicingly supported the hypothesis of separate clonal origins for the leukemic and myeloma components in this case. The correlation between multiple myeloma and myelogeneous leukemia was discussed.抄録全体を表示PDF形式でダウンロード (670K) -
稲垣 稔, 滝 正志, 富田 幸治, 山田 兼雄, 目黒 嵩1982 年 23 巻 11 号 p. 1727-1732
発行日: 1982年
公開日: 2009/01/26
ジャーナル 認証ありA 13-year-old girl with Bernard-Soulier syndrome had massive genital bleeding in her mearch. 5,800 ml of fresh blood and 108 units of platelet concentrates were transfused intensively for 6 weeks, but the bleeding was not improved. Then, progesteron. estrogen complex (Sophia A) was given to the patient, and the genital bleeding was effectively decreased.
In this report, her cliniccl course was followed up along its experimental trials concerning with its effects on platelet adhesion to subendothelium tested by Baumgartner's method as well as on platelet count, platelet aggregation and plasma antithrombin III level, and Sophia A was supposed to have little influence on platelet adhesion, platelet count, platelet aggregation or plasma antithrombin III level. The specific antibody to platelet membrane was examined to prove none of such antibody which was reported to exist after massive and multiple blood transfusions to cases of Bernard-Soulier syndrome.抄録全体を表示PDF形式でダウンロード (566K) -
名取 英世, 山口 達夫, 安田 佳織, 江上 康一郎, 滝井 優, 矢野 敬文, 田中 健, 加地 正郎, 白水 和雄1982 年 23 巻 11 号 p. 1733-1740
発行日: 1982年
公開日: 2009/01/26
ジャーナル 認証ありA 49-year-old man was admitted to the hospital because of gingival tumor and general weakness. Plasmacytoma was diagnosed by tumor biopsy. The white blood cell count was 5,400/cmm with 4% plasma cells. Bone marrow aspirations revealed 88.4% plasma cells.
Immunoelectrophoresis revealed monoclonal IgD (λ) in serum, and Bence Jones protein (λ) in urine. Quantitative immunoglobulin determination showed marked increase in IgD, 3,000 mg/dl.
He was diagnosed as leukemic IgD myeloma, and was treated with resulting in remission. After two months, he was readmitted because of continuous back pain.
One moth after the admission. he had bilateral pleural effusion. The thracenteses revealed bloody effusion with IgD, 1,250 mg/dl and numerous plasma cells. He was treated with Adriamycin and Methotrexate intrathracic, but died of sepsis eight months after the onset.
It was suggested that the pleural effusion resulted from infiltration of plasma cells to the pleura clinically, although an autopsy revealed no clear involvement of the pleura and no mass on the thorax.抄録全体を表示PDF形式でダウンロード (890K) -
陣内 逸郎, 雨森 龍彦, 吉田 善春, 松尾 辰樹, 栗山 一孝, 朝長 万左男, 上平 憲, 市丸 道人1982 年 23 巻 11 号 p. 1741-1749
発行日: 1982年
公開日: 2009/01/26
ジャーナル 認証ありA 14 year-old boy of typical cyclic neutropenia (CN) with 21 day cycle is reported. The bone marrow CFU-C concentration in this patient fluctuated from normal to very low values although it has been reported that CFU-C concentrations in most cases of CN fluctuate with in normal range or above upper lemit of normal values. This difference in fluctuation pattern of CFU-C concentration among CN cases means that there exists a heterogeneity in pathophysiology of cyclic granulopoiesis.
His serum colony stimulating activity (CSA) varied in parallel with the absolute monocyte count in peripheral blood. CSA of mononuclear leukocyte conditioned medium elevated coincidentally with peripheral monocytosis. These findings suggest that the fluctuation of serum CSA seems to be secondary to peripheral monocytosis.
Recently Dresch et al. proposed that cyclic granulopoiesis in CN may be caused by an inhibitor secreted by CN neutrophils. Assuming that a similar inhibitor is secreted from the neutrophils of our patient it may inhibit granulopoiesis at CFU-C level. It is very important to investigate the mechanism of granulopoietic inhibition by CN neutrophils and to clarify this inhibitory factor biochemically.抄録全体を表示PDF形式でダウンロード (643K) -
上平 憲1982 年 23 巻 11 号 p. 1750-1756
発行日: 1982年
公開日: 2009/01/26
ジャーナル 認証ありResults of serologic studies and clinical features of a patient with rheumatoid arthritis (RA) accompanied sicca complex and hyperviscosity syndrome whose serum contained extremely high serum levels of rheumatoid factor activity and pyroglobulinemia are described.
The patient, a 61 year old female Japanese, suffered in RA symptoms since 1952. The presence of abundant polyclonal IgG and monoclonal IgAK in plasma was detected by immunoelectrophoresis and single radial immunodiffusion. The intermediate complexes (9.9 S and 12.9 S) were demonstrated by ultracentrifugation and gel filtration analysis. The rheumatoid factor activity was attributed to a monoclonal IgAK paraprotein and titers of RAHA was of 1:40,960.
On the other hand, pyroglobulin of the serum was observed in inactivation (56°C, 30 minutes) in may 1981, but gel formation was diminished in the serum without IgG treated by staphylococcus aureus Cowan I strains. Recently, IgA paraprotein and RAHA titers were gradually decreased, and finally proglobulin was not observed in march 1982.
These data suggested that intermediate complex had the characteristics of forming precipitates at 56°C for 30 min.抄録全体を表示PDF形式でダウンロード (620K) -
岩瀬 滋, 宮下 ひろ子, 角尾 知砂, 高桑 俊文, 原 文男, 本田 光芳, 馬杉 洋三, 今村 幸雄1982 年 23 巻 11 号 p. 1757-1765
発行日: 1982年
公開日: 2009/01/26
ジャーナル 認証ありA 69 year old man was admitted to our hospital on Octover 16, 1976, because of general fatigue, fever, nasal and pharyngeal narrowing, general lymphnode swelling and skin rash.
On admission, physical examination revealed anemia, general skin rash and hepatosplenomegaly.
Laboratory examination showed hyper γ-globulinemia and M-components in serum and urine. These M-components were found to have M-bows only against AHWS and anti-γchain. Furthermore, the M-components in the urine were identified completely with γ-Fc (γ1) fragment by Ouchterlony technique. The molecular weights of the serum and urinary Fc fragment were presumed to be about 60,000 and 40,000∼50,000 by the immuno-thin layer chromatography (Sephadex G 200 superfine) respectively.
The histo-pathologic finding of the biopsy of the supraclavicular lymphnode revealed the destruction of normal architecture by the proliferated immunoblastic cell, lymphoid plasma cell, lymphocyte and plasma cell, which infiltrated partially in to the capsule. There are moderate proliferation of the small vessel. These findings resemble to the pathologic findings of the lymphnode of the angio-immunoblastic lymphadenopathy with dysproteinemia, and may be placed in the category of monoclonal immunoblastosis (Kojima). This case was a γ-heavy chain disease showing histological features similar to the angio-immunoblastic lymphadenopathy with dysporteinemia.抄録全体を表示PDF形式でダウンロード (1026K) -
坂巻 壽, 浜口 裕之, 間 栄, 斉藤 憲治, 榎原 英夫, 古沢 新平, 宍戸 英雄, 江口 光興1982 年 23 巻 11 号 p. 1766-1772
発行日: 1982年
公開日: 2009/01/26
ジャーナル 認証ありA case of congenital dyserythropoietic anemia type I (CDA-I) in a 72-year-old man was reported.
The patient had been pale since his chidhood but not visited any hospital till 1978, when he was found to have anemia and was treated with iron and vitamines without benefit. He was admitted to our hospital on September 24, 1980, to investigate the cause of anemia.
The RBC count was 1,940,000/cmm, hematocrit 20%, hemoglobin 6.4 g/dl, reticulocytes 2.1%, WBC 6,300/cmm with normal differential cell count, and platelets 210,000/cmm. A bone marrow aspirate revealed marked erythroid hyperplasia with bi- or multinucleation (9.2% of all erythroblasts) and internuclear chromatin bridges of erythorblasts (5.7%). Sideroblasts were 100% and 5.2% was of ringed form. δ-ALA synthetase activity of the marrow erythroblasts was low. Acidified serum test was negative. Ferrokinetic study showed the pattern of ineffective erythropoiesis. RBC life span was normal. The electron microscopic study of the bone marrow revealed conspicuous abnormalities compatible with CDA-I.
The identification of the disease at advanced ages, no evidence of inheritance and the presence of 5.2% ringed sideroblasts raised a suspicion of primary acquired sideroblastic anemia. However, the onset of anemia in this infancy, rarer incidence of ringed sideroblasts, no abnormalities in granulocyte and thrombocyte series, no reduction of the BFU-E and CFU-E, and the characteristic ultrastructural anomalies favor the diagnosis of CDA-I.抄録全体を表示PDF形式でダウンロード (1229K) -
田中 善紹, 海老沢 哲也, 李 利則, 沢田 昌平, 島崎 千尋, 中西 忍, 春山 春枝, 伊勢村 卓司, 中川 雅夫, 伊地知 浜夫1982 年 23 巻 11 号 p. 1773-1777
発行日: 1982年
公開日: 2009/01/26
ジャーナル 認証ありA case of AML with leukemic pleuritis as an initial manifestation and with leukemia cutis and meningeal leukemia as later complication was reported.
A 40-year-old male was admitted to our hospital on September 13, 1980 because of dyspnea and cough. Peripheral blood showed RBC 436×104/cmm, Hb 12.7 g/dl, plt 40.7×104/cmm and WBC 5,400/cmm with normal differential. A sternal bone marrow aspiration revealed N.C.C. 23.2×104/cmm with 52.6% leukemic cell. From positive peroxidase and chloroacetate esterase staining, the diagnosis of AML was made. The chest X-ray showed the bilateral pleural effusion and hillar lymph node swelling. The efftusion was exudative and contained many leukemic cells. A complete hematologic remission was achieved by DCVP therapy. Lymph node swelling and pleural effusion disappeared by successive two courses of consolidation therapy.
On Febrtary 23, 1981, the patient relapsed with leukemia cutis. At the end of March, the patient developed diplopia due to lt-abducens paralysis and was treated with four intrathecal injection of Ara-C, MTX and Pred. But remission was not reinduced in spite of intensive treatment. The leukemia cutis aggravated and the patient died of intracerebral hemorrhage in nine months survival.抄録全体を表示PDF形式でダウンロード (417K) -
平岡 諦, 池岡 清光, 高 義雄, 岩尾 憲人, 倉堀 知弘, 若宮 吉次郎, 金倉 譲, 玉置 俊治, 金山 良男, 垂井 清一郎1982 年 23 巻 11 号 p. 1778-1782
発行日: 1982年
公開日: 2009/01/26
ジャーナル 認証ありA 38-year-old man diagnosed as adult T-cell leukemia was described. He had watery diarrhea for two months and was isolated in our hospital because of the identification of Shigella sonnei. Despite the treatment with antibiotics sensitive to it, his symptoms worsend progressively. Blood examination revealed lymphocytosis; the most lymphocytes had deeply indented and lobulated muclei and rosetted spontaneously with sheep erythrocytes. Pseudopolyposis was revealed in the small and large intestine by barium enema and/or fiberscope. Light and electron microscopic exmainations of materials from large bowel revealed the infiltration of the leukemic cells into the entire mucous membrane. The treatment with dexamethasone and cyclophosphamide was unsuccessful.
Monoclonal antibodies characterized the phenotype of leukemic cells as helper/inducer T cells (namely OKT3+, OKT4+ and Leu 3a+). However the leukemic cells also reacted with OKT8 and Leu 2a, which are markers of cytotoxic/suppressor T cells.
The clinical and immunocytological chracteristics were discussed.抄録全体を表示PDF形式でダウンロード (687K) -
宮内 潤, 渡辺 陽之輔, 渡部 太郎1982 年 23 巻 11 号 p. 1783-1791
発行日: 1982年
公開日: 2009/01/26
ジャーナル 認証ありA 47-year-old Japanese woman was admitted to the hospital. On admission, splenomegaly and leukoerythroblastic anemia with poikilocysois including tear drop cells were noticed. Ph1-chromosome was negative. Bone marrow biopsy revealed hyperplasia of three blood cell lines, bizarre megakaryocytes, osteosclerosis, and slight fibrosis. A diagnosis of primary myelofibrosis was made. When she was 51 years old, splenic infarction occurred and splenectomy was performed. Marked extramedullary hematopoiesis was found in the spleen, biopsied liver and lymph nodes. Following a transient improvement of her blood picture, leukocytes with immature forms and erythroblasts increased in her blood. Many megaloblastoid cells and atypical erythroblasts with intense PAS reaction appeared. She died of disseminated fungal infection and miliary tuberculosis 1 year after the splenctomy. On autopsy, bone marrow showed leukemic hyperplasia and leukemic cells infiltrated into the liver, lymph nodes, uterus, ovaries, etc. Among leukemic cells, lysozyme- or hemoglobin-positive cells were demonstrated by immuno-histochemical staining. The terminal phase of her illness was diagnosed as erythro-leukemia. Concerning the diagnosis of leukemic transformation, the significance of erythroblastic abnormalities in course of primary myelofibrosis was discussed.抄録全体を表示PDF形式でダウンロード (1516K) -
西田 一弘, 津田 昌一郎, 前川 平, 枝川 潤一, 谷脇 雅史, 薗田 精昭, 沢井 公和, 三沢 信一, 阿部 達生, 瀧野 辰郎, ...1982 年 23 巻 11 号 p. 1792-1799
発行日: 1982年
公開日: 2009/01/26
ジャーナル 認証ありA 65-year-old female was admitted to our hospital because of persistent high fever, and remarkable hepatosplenomegaly. On admission abnormal cells (14%) were found in the peripheral blood. The diagnosis of leukemic B-cell leymphoma was made according to the hematological and immunological marker studies. Cytogenetic study was also revealed a pseudodiploid chromosome costitution with the presence of three consistent marker chromosomes, t(1;14)(p13;q22), t(1;9;16)(q21;q13;p13) and a medium-sized unidentifiable one. With DMA/DAPI staining, the latter was shown to have an extremely large and brightly fluorescent region which might be coincidental with the heterochromatic HSR described by Barker et al (1979).抄録全体を表示PDF形式でダウンロード (584K) -
米満 博, 金沢 正一郎, 近藤 春樹, 奥田 邦雄, 重田 英夫, 山口 覚太郎1982 年 23 巻 11 号 p. 1800-1807
発行日: 1982年
公開日: 2009/01/26
ジャーナル 認証ありA 45-year-old man admitted on December 1, 1980 because of easy fatigability and high fever. On admission, he had hepatosplenomegaly, lymphadenopathy and scattered petechae. Hematologic examination showed a moderate anemia, a white cell count of 222,000/mm3 with 94.5% lymphoblasts and marked thrombocytopenia. Bone marrow aspiration revealed 98% lymphoblasts. Their surface marker study yielded E rosettes formation in 2% and EAC rosettes formation in 2%. The terminal deoxynucleotidyl transferase activity by the immunofluorescent assay was elevated. The karyotype of the bone marrow cells was 46, XY, t(9q+; 22q-). He was diagnosed as haaving Ph1 positive acute lymphoblastic leukemia or lymphoid blast crisis at the onset of chronic myelogenous leukemia (CML). He was treated with viencristine, Endoxan, 6MP and prednisolone (VEMP), and a complete remission was achieved 3 months after admission. Subsequently, the peripheral blood showed changes similar to those in the chronic phase of CML. In April, 1981, the number of lymphoblasts in the peripheral blood and in the bone marrow rapidly increased and the patient was readmitted. He became febrile with septecemia due to E. coli and Pseudomonas, and died on June 30, 1981.
At autopsy, infiltration of lymphoid cells was seen in the bone marrow, spleen, liver, kidneys, lymph nodes, lungs, and protoate. The past reports on the cases with CML presenting as acute leukemia at onset and Ph1 positive acute leukemia have been reviewed.抄録全体を表示PDF形式でダウンロード (656K) -
山谷 敏彦, 鎌田 義正, 永井 一徳, 沢田 美彦1982 年 23 巻 11 号 p. 1808-1815
発行日: 1982年
公開日: 2009/01/26
ジャーナル 認証ありA case of the so-called Chronic Neutrophilic Leukemia is reported.
A 65-year-old male was admitted on October 1980, because of marked neutrophilia and hepatosplenomegaly. On admission, his hemoglobin level was 9.6 g/dl, the platelet count 18.1×104/cmm and the leukocyte count 38,000/cmm. Eighty-seven percent of leukocytes were mature neutrophile leukocyte-like cells and neither eosinophile nor basophile leukocytes were seen.
The leukocyte alkaline phosphatase score was 380, serum vitamine B12 was 6,030 pg/ml, and urine as well as serum muramidase level was markedly elevated.
Bone marrow aspiration and biopsy revealed a hypercellular marrow with hyperplasia of myeloid elements.
A karyotypic analysis showed a 46, XY pattern with no evidence of the Ph1 chromosome. Colony forming unit in culture of this neurtrophile leukocyte-like cell was much decreased in both peripheral blood and bone marrow.
Examined by the transmission electron miroscope, leukemic cells revealed a segmented nucleus with marked chromatin condensation and multilamellae of the rough-surfaced endoplasmic reticulum arranged in a parallel manner along with a well developed Golgi apparatus in the cytoplasm. These fidings were thought to indicate asynchrony between the developement of the nucleus and cytoplasm.
Despite an intensive chemotherapy, he died from dyspnea on September 16, 1981. Pstomortem examination revealed marked proliferation of myeloid cells in the bone marrow, splenomegaly (850 g) and hepatomegaly (2,000 g) with infiltration of myeloid elements.抄録全体を表示PDF形式でダウンロード (906K) -
唐沢 正光, 沢村 守夫, 岡本 克実, 岩田 展明, 小峰 光博, 前川 正, 杉原 尚, 吉本 正博, 八幡 義人1982 年 23 巻 11 号 p. 1816-1822
発行日: 1982年
公開日: 2009/01/26
ジャーナル 認証ありA 18-year-old Japonese girl with congenital hemolytic anemia associated with elevated red cell membrane phosphatidyl choline and enhanced Na+-transport is described. At the age of 4, she was diagnosed as having congenital hemolytic anemia after detailed examination. She was hospitalized twice thereafter because of persistent jaundice and repeated abdominal pain before undergoing splenectomy and cholecystotomy at the age of 14 with a tentative diagnosis of hereditary spherocytosis. Her hemolytic anemia however did not improve appreciably. Because of recurrent cholelithiasis, the cholecystectomy was performed at 18 years of age. Soon thereafter she was referred to us for further investigation. Her Hb was 11.3 g/dl, reticulocytes 29.3% and WBC 7,700/ul. A moderate macrocytosis (MCV 108μ8) with target cells was noted. Her bilirubin was 10.5 mg/dl with 8.3 mg/dl indirect-reacting bilirubin The osmotic fragility was decreased and autohemolysis showed Dacie's type I pattern. Hemoglobin analysis and red cell enzyme activities were unremarkable. Her red cell membrane showed increased cholesterol and phospholipids per RBC along with elevated phosphatidyl choline (33.0%) and depressed phosphatidyl ethanolamine (25.3%). Sodium transport was markedly elevated. Her parents and two siblings were all normal with respect to the red cell lipids and sodium transport. There were no similar diseases in her family tree. Thus, her hemolytic anemia appeared to be related to the abnormal red cell lipid constitution, analogous to that reported by Jaffé et al as high phosphatidyl choline hemolytic anemia.抄録全体を表示PDF形式でダウンロード (559K) -
長谷川 吉康, 網野 皓之, 中辻 理子, 三輪 史朗, 宮地 隆興, 大庭 雄三1982 年 23 巻 11 号 p. 1823-1828
発行日: 1982年
公開日: 2009/01/26
ジャーナル 認証ありAn electrophoretically silent unstable hemoglobin was found in a 23-year-old woman suffering from hemolytic jaundice and anemia. The unstable β subunit was precipitated by p-chloro-mercuribenzoic acid and globin was further purified by urea CM-52 colum chromatography. Results of amino acid analysis, tryptic fingerprinting and manual Edman degradation of the abnormal βT12B established substitution of proline for histidine β117 (G 19).
This hemoglobin exhibited low oxygen affinity and high content of 2, 3 DPG in blood as compared with normal control. The abnormal hemoglobin seems to have arisen by de novo mutation, because of absence in other members of the family. This new mutant hemoglobin β117 (G 19) His→Pro named Hb Saitama, in realation to her present address.抄録全体を表示PDF形式でダウンロード (413K)
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