臨床血液
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
31 巻, 9 号
選択された号の論文の29件中1~29を表示しています
臨床研究
  • 相馬 正幸, 前田 義久, 笠倉 新平, 矢部 博樹
    1990 年 31 巻 9 号 p. 1445-1449
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    In order to define a specific acceleration of fibrinolytic activity in acute promyelocytic leukemia (APL), we determined fibrinolytic factors in APL and actue myeloblastic leukemia (AML).
    An increase in plasma levels of D-dimer was observed in both APL and AML, indicating that there is an acceleraton of fibrinlysis in both types of leukemia. The levels of D-dimer/FDP ratio were significantly lower in APL than AML. These findings suggest that fibrinogenolytic activities were higher in APL that in AML.
    The relationship between the plasma levels of plasmin α2PI complex (PIC) and FDP was investigated to study whether fibrinolysis was induced by plasmin. PIC levels were linealy correlated with FDP levels in AML, while in APL there was no close correlation between the plasma levels of PIC and FDP. Then, we measured PMN elastase-α1 proteinase inhibitor complex (E-α1PI). There was a correlation between the plasma levels of E-α1PI and FDP in APL but not in AML. Furthermore, PMN elastase activity was detected in leukemic cell lysate in patients with APL but not in AML.
    These findings suggest that PMN elastase may be an important factor in the inducton of fibrinolysis in APL.
  • 中尾 真二, 大竹 茂樹, 塩原 信太郎, 森 孝夫, 松田 保
    1990 年 31 巻 9 号 p. 1450-1455
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    We treated 14 patients with aplastic anemia (6 severe, 8 moderate) who did not respond to high dose methylprednisolone, antilymphocyte globulin, or anabolic steroid with cyclosporin (CyA) for more than 4 weeks. Four of them (29%) showed clinical improvement to transfusion-independence. The response rate of patients who received CyA more than 5 weeks was 50% (4 out of 8). The dose of CyA given to the 4 patients who improved after the therapy was 5 to 9 mg/kg/day. One patient needed 11 weeks until the first sign of response (increase of reticulocytes) appeared. In the other three patients, platelets increased first in response to CyA within 5 weeks. Side effects such as hypertricosis and gingival hyperplasia were frequently seen during the CyA therapy, but since they were not so severe or transient, almost all patients tolerated the therapy. These results indicate that CyA can be an efficacious drug for patients with refractory aplastic anemia. Long term treatment with a relatively low dose of CyA may be important for obtaining a high response rate.
  • 浅野 茂隆, 平嶋 邦猛, 吉田 彌太郎, 高久 史麿, 宮崎 保, 小峰 光博, 古沢 新平, 阿部 帥, 森 眞由美, 有森 茂, 阿部 ...
    1990 年 31 巻 9 号 p. 1456-1462
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    The effect of recombinant human granulocyte colony-stimulating factor (rhG-CSF) on neutropenia was studied in 30 patients with aplastic anemia (AA). RhG-CSF was administerd intravenously daily at a dose of 2, 5, 10, or 20μg/kg/day for more than 7 days.
    In the patients whose absolute neutrophil counts (ANC) were more than 0.1×109/l, the rhG-CSF injections at ≥5μg/kg/day caused rapid and selective elevation of ANC which maintained during the injection period.
    Most of the patients were well tolerated, and minor side effects were observed in only 3 patients.
    These findings suggest that daily injections of rhG-CSF at a dose of ≥5μg/kg/day may be an effective strategy for the treatment of bacterial and/or fungal infections in AA patients.
症例
  • 新名主 宏一, 岡留 敏秀, 丸山 芳一, 丸山 征郎, 納 光弘
    1990 年 31 巻 9 号 p. 1463-1467
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    A family with two complex disorders of hemostasis, von Willebrand disease (vWD) and hypofibrinogenemia was reported.
    The probands were 21-and 16-year-old full brothers suffering from serious bleeding tendencies from childhood. The elder brother had a subarachnoid hemorrhage at the age of 15. The younger brother had repeated episodes of gastrointestinal bleedings since he was 10 years of age. Coagulation studies revealed that both of them had almost the same hemostatic abnormalities, i.e. severe vWD, 9 to 12% of plasma vWF levels, and mild hypofibrinogenemia, 125 to 130 mg/dl of plasma fibrinogen levels. Multimeric compositions of their vWF were normal, and functional assay for fibrinogen concentration yielded essentially the same values as did immunologic assay. These results indicated that they had two complex disorders, extreme type I vWD and heterozygous state of afibrinogenemia resulting in serious bleeding tendency. Family study showed that these two hemostatic disorders were paternal inheritance, and it was strongly postulated that vWD and hypofibrinogenemia might be highly-combined hemostatic disorders
  • 新名主 宏一, 太良 光利, 岡留 敏秀, 丸山 芳一, 丸山 征郎, 納 光弘
    1990 年 31 巻 9 号 p. 1468-1473
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    We report a case of pure red cell aplasia (PRCA) with benign monoclonal gammopathy (BMG) of IgA·λ type and type I von Willebrand disease (vWD).
    A 61-year-old female patient was treated initially with prednisolone, azathioprine and cyclophosphamide with transient and unsatisfactory reticulocyte response. Oral administration of 200 mg of cyclosporine A (CyA)/day was started from July, 1987. A rapid and marked reticulocytosis was seen from a week later and there was a rapid increase in hemoglobin levels, and remission has been maintained for over 22 months.
    Patient's serum and IgA taken on admission did not show inhibitory activity to both CFU-E growth from her own bone marrow cells obtained in remission and von Willebrand factor. T cell-mediated suppression to CFU-E growth was detected. On family study, the patient's second son was found to be a type I vWD.
    These results indicate that there is no direct causal relationships between BMG and PRCA or vWD, and that CyA may have a place in the management of PRCA.
  • 高屋 正敏, 市川 幸延, 有森 茂
    1990 年 31 巻 9 号 p. 1474-1477
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    Successful pregnancy outcome is described in a patient with hemoglobin E/β°-thalassemia which is expressed as thalassemia major.
    A 21 year-old Laotian female complicated by severe anemia became pregnant. The diagnosis of hemoglobin E/β°-thalassemia was made by hemoglobin analysis. The fetal weight estimated by abdominal echogram decreased during pregnancy. She received blood transfusions, in order to improve intrauterine growth retardation. Hemoglobin concentration was maintained at the level greater than 9g/dl. Fetal growth was improved by the treatment. A normal full-term male infant was born by a caesarean section, and splenectomy was performed at the same time.
  • 日野 理彦, 山辺 高司, 小野 哲也, 佐々木 なおみ, 難波 紘二
    1990 年 31 巻 9 号 p. 1478-1482
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    Systemic mast cell disease (SMCD) is a rare disease often associated with symptoms of general malaise, pruritis, diarrhea, vomiting, fever, urticaria pigmentosa, hepatosplenomegaly and lymphadenopathy.
    We reported a case of SMCD associated with cutaneous xanthoma and serum hyper IgE. Skin biopsy revealed xanthomas and diffuse infiltration of mast cells in the dermis. The association of SMCD with xanthoma was reported in the literature for only one case. The hyper IgE could be due to the defect of IgE receptors on the cell membrane of mast cells or dysfunction of T and/or B cell. Any of the treatment using H1 and H2 receptor blockade, disodium cromoglycate, adrenocorticosteroid or chemotherapy (VEPA) were not effective. The patient died of pulmonary edema and multiple organ failure 7 months after the diagnosis was established.
    The crush method for the cytological examination of bone marrow was considered more useful than smear method for the diagnosis of SMCD.
  • 木村 之彦, 倉辻 忠俊, 伊藤 良和, 桑原 三郎, 川西 慶一, 児島 辰也, 外山 圭助
    1990 年 31 巻 9 号 p. 1483-1488
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    A 19-year-old male was diagnosed as having chronic granulomatous disease (CGD) based on negative NBT reduction in January, 1988. He was admitted with a chief complaint of high fever in November, 1988. As abdominal echogram and CT scan established a diagnosis of multiple hepatic abscesses, he was treated with various kinds of antibiotics. Since the therapy was ineffective, the number of circulating neutrophils was decreased, and the abscesses further grew, intravenous drip infusion of rhG-CSF 100 μg was initiated in addition to several antibiotics (sulfamethoxazole-trimethoprim, rifampicin, isoniazide, etc). At day 3 on rhG-CSF, the fever began to resolve and on day 15 the body temparature fell below 37°C. The hepatic abscesses also tended to decrease in size and the CT scan performed 2 months later (March 17), disclosed only calcification in the liver. The neutrophil function test indicated that superoxide anion (O2-) and hydrogen peroxide (H2 O2) production was slightly increased during rhG-CSF therapy. Combination therapy with rhG-CSF and potent antibiotics showed a favorable therapeutic effect on CGD complicated by multiple hepatic abscesses as a fatal infection.
  • 吉村 祐一, 中山 明子, 浜 英永, 小口 淳, 松田 重三, 鎌倉 正英, 木下 忠俊, 山中 正巳, 安部 英
    1990 年 31 巻 9 号 p. 1489-1492
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    Glucocorticoid and androgen therapy have been used with moderate success in paroxysmal nocturnal hemoglobinuria (PNH). However, both are poorly tolerated, especially in women, although the side effects of glucocorticoid can be diminished by alternate day therapy. We have treated two patients with PNH with danazol. One of patient is man, 64 years old age with stomach cancer, and the other patient is 34-year-old man. Their disease has existed for 6-14 years. They required many blood transfusions, their hemoglobins ranging between 7.1 and 9.9 grams per deciliter.
    When treated with danazol by mouth, the hemoglobin level increased approximately 2 to 5 grams in each patient within 3 week, and clinical hemoglobinuria improved. None of the patients has had any side effects.
  • 石丸 文彦, 林 久智, 藤田 敏明, 鶴見 尚和, 津田 隆史, 木村 郁郎
    1990 年 31 巻 9 号 p. 1493-1498
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    Acquired von Willebrand syndrome is reported in a 31-year-old woman with autoimmune hemolytic anemia (AIHA). The patient, who had no family history of bleeding, presented with a hemorrhagic diathesis of recent origin. Routine coagulation studies showed a normal platelet count, prolonged bleeding time and abnormal glass bead retention Plasma levels of factor VIII-von Willebrand factor (F VIII-vWF) were low and ristocetin-induced platelet aggregation (RIPA) was decreased. In vitro, the patient's plasma exhibited inhibitory activity against vWF: ristocetin cofactor activity (vWF: Rco) but had no effect on RIPA. The multimeric pattern of the patient's vWF resembled that of patients with inherited type II von Willebrand disease, in that the largest multimers were missing. Clinical improvement resulted after treatment of AIHA with prednisolone (PSL), and F VIII-vWF returned to normal levels with normalization of vWF multimers. However, following tapering the dose of PSL, plasma levels of F VIII-vWF again decresed.
  • 井上 信正, 武元 良整, 岡本 隆弘, 神前 昌敏, 金丸 昭久, 垣下 榮三, 三澤 真人, 大江 与喜子, 甲斐 俊朗, 原 宏, 杉 ...
    1990 年 31 巻 9 号 p. 1499-1505
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    Five patients with myelodysplastic syndrome (RA: 4 cases, RAEB in T: 1 case) were treated with myeloablative immunosuppressive therapy followed by bone marrow transplantation (BMT).
    Median age was 20-y-o (11∼31-y-o). All patients were prepared with cyclophosphamide and total body irradiation. Engraftment was documented in all patients. One patients (case 4, 31-y-o female) died of brain hemorrhage due to the thrombocytopenia refractory to platelet transfusion because of anti-platelet antibody in 34 days after BMT. A patient with RAEB in T was also died of respiratory failure from interstitial pneumonia on Day 173. One patient (case 1, 22-y-o, female) progressively became granulocytopenic and thrombocytopenic status after BMT. She suffered from life-threatening infection and then received a second bone marrow cell infusion from the same donor without any preparative conditioning.
    These results suggest that BMT could be the treatment of choice for MDS, especially for the patients with RA who have poor prognostic factors including life-threatening cytopenia and or cytogenetical abnormalities.
  • —症例報告と文献的考察—
    酒井 力, 南久松 真子, 高木 敏之, 小黒 昌夫, 丸山 孝士, 田中 公夫, 鎌田 七男
    1990 年 31 巻 9 号 p. 1506-1511
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    A 60-year-old woman was admitted because of fatigue. Physical examination revealed prominent peripheral lymphadenopathy, marked tonsillar swelling and hepatosplenomegaly. The leukocyte count was 68,900/μl with 75% lymphoid blasts and 5% basophils. The karyotype of the blood cells was 46, XX, Ph1/47, XX, Ph1, +Ph1. The diagnosis of CML in blast crisis was made. After chemotherapy using adriamycin, cyclophosphamide, vincristine, and prednisolone (CHOP), lymphadenopathy and splenomegaly reduced and lymphoid blasts disappeared from the blood and bone marrow. At that time only single Ph1 (46, XX, Ph1) clone was detected in her bone marrow. Four months later, hematological relapse accompanied by lymphadenopathy occurred and DNA analysis of the blasts showed the rearrangement of bcr gene. The simultaneous chromosomal analyses of the blood, bone marrow and lymph node revealed that almost all cells examined had the karyotype “47, XX, Ph1, +Ph1”. In spite of repeated chemotherapy the patient did not improve and died.
    This case suggests a relationship between lymphadenopathy and double Ph1 chromosomes in CML.
  • 楠本 修也, 松田 晃, 福田 正高, 陣内 逸郎, 別所 正美, 斎藤 昌信, 平嶋 邦猛
    1990 年 31 巻 9 号 p. 1512-1516
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    A 46-year-old woman was admitted to our hospital because of leukocytosis. A diagnosis of acute lymphoblastic leukemia (FAB: L2 type) was made by reviewing peripheral blood smear and bone marrow aspirate. Chromosome analysis showed the presence of Philadelphia chromosome. A combination chemotherapy with L-asparaginase, doxorubicin, vincristine, and prednisolone was started, but complete remission was not achieved. During a neutropenic period after combination chemotherapy with doxorubicin, vincristine, vinblastine, and VP-16, high fever and tender swelling of the right cheek were noticed. A diagnosis of maxillary sinusitis was made with tomography and CT scan of the maxillary sinus. Since culture of the aspirate from the maxillary sinus grew aspergillus, a diagnosis of aspergillosis of the maxillary sinus was made.
    Immediately after the intravenous administration of amphotericin B and the lavage of the sinus with amphotericin B was started, high fever subsided and clinical improvement was observed. Several regimens of chemotherapy failed to obtain hematological remission, she died of sepsis of Enterobactor cloacae without evidence or relapse of dissemination of aspergillosis after initial successful treatment.
    While a few cases with aspergillus maxillary sinusitis were reported in leukemic patients, the possible occurrence of this complication must be kept in mind in a severe neutropenic period after intensive chemotherapy.
    The combination of intravenous administration and local lavege of amphotericin B appeared to be an effective treatment in the Aspergillus maxillary sinusitis.
  • 久保田 徹, 岡村 孝, 林 真, 永渕 正法, 大塚 輝久, 渋谷 恒文, 原田 実根, 仁保 喜之
    1990 年 31 巻 9 号 p. 1517-1522
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    A 38-year-old female with acute lymphoblastic leukemia developed monoplegia of the left upper extremity following chemotherapy for remission induction consisting of vincristine, prednisolone, cyclophosphamide, adriamycin and methotrexate. Hemorrhagic infarction due to thrombosis of the right cortical vein was diagnosed by brain images using computed tomography and magnetic resonance imaging in addition to the clinical course. At this time, a plasma level of fibrinogen measured by the thrombin time method had decreased to 84 mg/dl, while its antigenicity was 276 mg/dl. There was no evidence of activation of the blood coagulation and fibrinolysis system nor of abnormal liver function. A mixing test with normal plasma disclosed the absence of an inhibitory factor against fibrin polymerization in her plasma. Fibrinogen levels as assessed by the thrombin time method recovered to the antigenicity level one and half months later. The discrepancy between the activity and antigenicity of fibrinogen indicated the occurrence of acquired dysfibrinogenemia, probably induced by antileukemic agents. Thus, it is suggested that dysfibrinogenemia is a possible cause of cerebral thrombosis in this patient.
  • 西井 一浩, 片山 直之, 駒田 文彦, 影山 慎一, 星野 賢一郎, 塚田 哲也, 西川 政勝, 小林 透, 南 信行, 白川 茂
    1990 年 31 巻 9 号 p. 1523-1526
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    A 57-year-old woman was admitted to our hospital because of further examination of anemia in November, 1978. She was diagnosed as having aplastic anemia, which was not associated with any atypical findings. Treatment with oxymetholone had been effective. However, she developed pancytopenia in January, 1988. A bone marrow aspiration revealed a hypocellular marrow with 75.6% blasts. Following two courses with low dose cytosine arabinoside and vitamine D3, the leukemia improved although she needs red cell transfusions at intervals of one to two months because of persistent pancytopenia. We present a patient with aplastic anemia who developed a hypoplastic leukemia at the tenth year of the disease.
  • 岩本 一郎, 岩永 隆行, 入交 清博, 堀内 篤
    1990 年 31 巻 9 号 p. 1527-1531
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    A 33-year-old woman was hospitalized because of bleeding tendency. Hemoglobin was 10.7 g/dl, white blood cell 2,100/μl and platelet 2.1×104l. Bone marrow showed marked dysplasia of trilineage blood cells. Atypical blasts and monocytoid cells accounted for 14.5% in the myelogram. Cytogenetic study of bone marrow cells revealed translocation with t(11;21) in all of 20 metaphasic cells analyzed by G-banding method. A diagnosis of RAEB was made. Familial survey revealed that her elder brother died of acute monocytic leukemia (AMoL). The patient received small dose therapy of Ara-C and BHAC-DMP therapy, but a remission was not obtained. The patient's general condition deteriorated with infection, bleeding tendency and chronic hepatitis due to transfusions, therefore we have followed up the patient with prednisolone and red blood cell transfusion.
    It has become evident that some types of acute leukemia with monocytic features have a cytogenetic change at 11 q 23. But it is rare that RAEB with increased monocytoid cells has a cytogenetic change at 11q23. In addition, the patient's elder brother died of AMoL. This case is important in relation to cytogenetic change at 11q23 and hematopoietic abnormalities.
  • 中村 正, 穂満 直子, 児玉 千早, 大坪 秀雄, 日高 史郎, 梶原 裕二, 大徳 恭久, 有馬 直道, 松下 格司, 福盛 順子, 坂 ...
    1990 年 31 巻 9 号 p. 1532-1536
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    A 30-year-old female was admitted to our hospital complaining of hematuria and right flank pain in September, 1987. She had been diagnosed idiopathic thrombocytopenic purpura in 1980, and had similar symptoms before. Hematoma in the right ureter was demonstrated by retrograde pyelography and CT-scanning, and these symptoms improved within one month. Each activity of plasma clotting factors was within normal limits. Enzymatic studies of the urine revealed low values of plasmin-, urokinase-, and kallikrein-like activities in both excerbation and remission.
    These hemorrhagic tendencies might have been the result of marked thrombocytopenia: After bleeding into the urinary tracts began, the bleeding would tend to form hematoma because of elevated clotting activity; then hematoma would grow due to decreased urine fibrinolytic activities. This suggested that a decline of fibrinolysis in urine might have a promoting effect on the process of hematoma formation.
  • —特に血中サイトカイン値について—
    和田 英夫, 西塔 正広, 玉木 茂久, 桝屋 正浩, 森田 孝一, 田畑 三奈, 谷川 元昭, 影山 慎一, 辻 幸太, 大田 千鶴子, ...
    1990 年 31 巻 9 号 p. 1537-1543
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    We studied 10 patients with thrombotic thrombocytopenic purpura (TTP) and 5 patients with hemolytic uremic syndrome (HUS). Common cold symptoms were observed in 2 with TTP and 3 with HUS, and SLE was noted or suspected in 3 with TTP, and the onset was after operation in on with TTP and one with HUS. All TTP patients had coma and high fever. Renal failure was noted in 3 with TTP and their prognosis was poor. Seven patients with TTP and 4 patients with HUS survived. Autoantibody was highly positive in TTP but slightly positive in HUS. High molecular weight multimer of von Willebrand factor was decreased in 3 of 6 with TTP, platelet aggregating factor was positive in 4 of 6 with TTP, and microthrombus was observed in 7 of 8 with TTP. Tumor necrosis factor was increasd in 5 of 9 with TTP and HUS, Interleukin-1β was increased in all TTP and HUS patients, and soluble interleukin 2 receptor and interferon α were also increased. Although plasma exchange was generally effective, some patients required combination therapy with steroids. We speculated that an autoimmune mechanism was involved in the on onset of TTP.
  • 樋口 光宏, 紺屋 浩之, 田中 弘子, 増田 浩史, 西田 恭治, 小山 哲司, 末廣 謙, 垣下 榮三, 井下 勝男
    1990 年 31 巻 9 号 p. 1544-1547
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    Pregnant women with hereditary antithrombin III (AT-III) deficiency are frequently associated with thromboemblic disorders. We have treated a pregnant woman with hereditary AT-III deficiency, who had suffered from thromboemblic disorders at her past three gestations, with AT-III concentrate. Dosage of AT-III concentrate to maintain plasma AT-III activity over 80% was 3,500 units per week during second and third trimesters, but more frequent administration was necessary around delivery. In recent reports, pregnant women with hereditary AT-III deficiency had been treated with heparin or warfarin except for during abortion and delivery, in which time AT-III concentrate was widely utilized. But the use of heparin or warfarin during gestation is occasionally harmful, AT-III concentrate should be chosen for management in pregnancy in women with hereditary AT-III deficiency.
  • 中村 秀男, 渡部 富廣, 林田 孝子, 市丸 道人
    1990 年 31 巻 9 号 p. 1548-1552
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    In October 1988, a 72-year-old man was admitted to Nagasaki Prefectural Shimabara Onsen Hospital because of hemoglobinuria on exposure to cold. On admission, the laboratory data were Hb 9.1g/dl, LDH 2,337U/L, and haptoglobin<6mg/dl. The Donath-Landsteiner (DL) test was positive and serological tests for syphilis were negative. Anti-Coxsackie virus type A9 antibody titer was elevated. Accordingly, he was diagnosed as having paroxysmal cold hemoglobinuria (PCH) associated with viral infection. The DL antibody proved to be of the IgM class having anti-I specificity. The patient was treated unsuccessfully with prednisolone, but hemolysis was improved after azathioprine (AZP) therapy.
    PCH patients with DL antibody of the IgM class exhibiting anti-I specificity are very rare and only two patients including the present case have been reported in Japan. There has been no report on the efficacy of AZP in PCH, but our study suggests that AZP may have a potential therapeutic effect in some PCH patients.
  • 上村 由樹, 岩原 義人, 片岡 隆策, 宗石 秀典, 三好 勇夫
    1990 年 31 巻 9 号 p. 1553-1556
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    Idiopathic myelofibrosis is a disease of unknown cause characterized by systemic marrow fibrosis and extramedullary hematopoiesis. We report here a patient of myelofibrosis treated successfully by busulfan pulse therapy which was reported first by chang et al in 1988. The patient was a 62-year-old woman who was referred to us for anemia and hepatosplenomegaly in August 1984. Further examination established a diagnosis of idiopathic myelofibrosis. During the subsequent 4-year follow-up period without specific treatment in our outpatient clinic, there occurred gradual progression of anemia and hepatosplenomegaly with the spleen extending beyond the level of the umbilicus. In September 1988, she was initiated on 4-day pulse therapy of busulfan with a daily dose of 12 mg, which was repeated 10 times until July 1989. This was followed by marked improvement of anemia and hepatosplenomegaly. Post-treatment iliac marrow biopsy showed some reduction of reticulin fibers with increased hematopoietic elements as compared to pretreatment iliac marrow biopsy. The busulfan pulse therapy, therefore, appears to be a treatment of choice in idiopathic myelofibrosis.
  • 永井 雅巳, 池田 和真, 田所 久徳, 徳田 道昭, 田坂 大象, 田中 輝和, 入野 昭三
    1990 年 31 巻 9 号 p. 1557-1560
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    The adult intussusception is rare. Here, we describe a case of an adult patient with ileocolic intussusception secondary to a recurrent lymphoma of the terminal ileum.
    The patient was a 42-year-old female, who had a history of the subtotal gastrectomy because of the primary gastric lymphoma. Eight months after the operation, she had a relapse in the abdomen and received the combination chemotherapy. She acquired the complete remission, but six months after the completion of the chemotherapy, she suffered from the right lower abdominal pain and diarrhea. Physical examination revealed a soft mass with tenderness in the right iliac fossa. Barium studies and abdominal computed tomography showed an ileocolic intussusception. At laparotomy, a 4.5 cm polypoid tumor was found in the ternimal ileum and it passed through the ileocaecal valve to form an intussusception. Histologic examination disclosed the follicular lymphoma of medium-sized cell type.
    So far as we are aware, this is the first report of an adult patient with intussusception secondary to a lymphoma in Japan.
  • 橋本 佳巳, 藤田 浩之, 菅村 リカ, 田辺 寿一, 藤沢 信, 野口 太平, 松崎 道男, 毛利 博, 大久保 隆男, 原 正道
    1990 年 31 巻 9 号 p. 1561-1565
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    The biopsy findings of malignant lymphoma involving the pericardium in a 69-year-old male were reported. The initial presenting symptoms were heart failure followed by rapidly progressive complete heart block. Pacemaker was inserted. Chest roentgenogram showed a marked left pleural effusion. An echocardiogram demonstrated pericardial fluid. The diagnosis of lymphoma was suggested by gallium isotope study with markedly increased isotope uptake in heart, and was subsequently confirmed by pericardial biopsy. After chemotherapy the pleural effusion was decreased and the uptake of gallium disappeared.
    While the diagnosis of cardiac involved lymphoma was hard antemortem, experience in this case suggests that gallium isotope study and pericardial biopsy are helpful in establishing the correct diagnosis.
  • 新津 秀孝, 高津 洋, 三浦 偉久男, 中鉢 明彦, 伊藤 俊広, 広川 誠, 遠藤 安行, 三浦 亮, 福田 光之, 佐々木 毅
    1990 年 31 巻 9 号 p. 1566-1571
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    We report a patient who had abrupt onset of pure red cell aplasia (PRCA) induced by B19 parvovirus during allogeneic bone marrow transplantation (BMT).
    A 14-year-old girl with APL in complete remission was admitted in February 1988, for the purpose of BMT. She was received marrow from HLA identical sister on March 17, 1988 (day 0). She received 120 mg/kg cyclophosphamide and 12 Gy total body irradiation for conditioning of BMT. For graftversus-host disease (GVHD) prophylaxis she was given cyclosporine and short term methotrexate. She did not develop acute GVHD after BMT, but on the day 28 a bone-marrow aspirate revealed findings of PRCA. During this course the number of white blood cell and platelet favorably recovered. B19 parvovirus DNA was detected in the serum of the day 30 and day 42. Antihuman B19 parvovirus (HPV) antibody titers were increased: the values of anti-HPV IgM were suddenly elevated and those of anti-HPV IgG were elevated. Serum on the day 42 inhibited erythroid progenitors (CFU-E, BFU-E) but not inhibited myeloid progenitors (CFU-C). A reticulocyte count recovered on the day 50. As the patient was HPV-IgG negative prior to BMT and the donor was HPV-IgG seronegative, the source of infection may be platelet tranfusion (day 7 through 14).
  • 田辺 義博, 安 恵美, 二木 真琴, 伊藤 敏治, 猪口 孝一, 山田 隆, 五味 聖二, 大木 一郎, 桑原 哲夫, 檀 和夫, 厨 信 ...
    1990 年 31 巻 9 号 p. 1572-1575
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    A 59 years old woman, born in Fukuoka Prefecture, was admitted to our hospital in Aug, 1988 because of diarrhea, fever and skin eruption. Physical examination revealed systemic lymphadenopathy and hepatosplenomegaly. The white blood cell count was 11,200/μl with 28% atypical lymphocytes with convoluted nuclei. Mild anemia, thrombocytopenia and hypercalcemia were also observed. Antibody against the adult T-cell leukemia (ATL) associated antigen in serum was positive. OKT 4/8 ratio was high. A diagnosis of ATL was made. Because of the complications of pneumonia and herpes simplex, systemic chemotherapy was not given, and interferon (IFN)-alpha-2b was intramusclaly injected daily from Oct, 1988, resulting in the disappearance of atypical lymphocytes and improvement of skin lesions. The effect of IFN therapy lasted for three months, followed by increase of atypical lymphocytes. Although the patient became refractory to systemic IFN therapy, local injection of IFN into a buccal tumor infiltrated with atypical lymphocytes resulted in its regression of size. In spite of continued administration of IFN, the patient died of pneumonia in Jan, 1989.
  • 新井 望, 原 明博, 梅田 正法, 白井 達男
    1990 年 31 巻 9 号 p. 1576-1580
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    The patient was a 47-year-old male who attended our hospital complaining of back pain. Masses in the bilateral adrenal glands were noted on abdominal ultrasonography and the patient was admitted to the hospital on August 9, 1988. Upon test laparotomy, non-Hodgkin's lymphoma (diffuse small-cell type, B-cell type) was diagnosed, and endocrinological examination revealed hypofunction of the adrenal gland. The results of various tests showed absence of distance metastasis except for that to the bilateral adrenal glands. Since complete remission was attained after 2 courses of COP-BLAM III therapy, and adrenal function also became normal, the patient was discharged on January, 9, 1989.
    Malignant lymphoma originating from the adrenal gland seems to be a rare condition, and only 16 cases have been reported to this date.
短報
  • 大久保 進, 石田 萠子, 安永 幸二郎
    1990 年 31 巻 9 号 p. 1581-1582
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    IgG subclasses (G-sub) of warm red cell autoantibodies (anti-Rs) were examined by means of antiglobulin test (DAT) using anti-IgG1, 2, 3, and IgG4 sera (Holland Red Cross) on 12 AIHA, and on 5 cases being DAT positive caused by α-methyldopa (αMD). In 4 of 5 AIHA cases complicating SLE, the anti-Rs comprised not only IgG, but also IgA, IgM and C3; their G-sub were IgG1+2 in 2 and IgG1+2+3+4 in the other 2. In all of the 3 cases with idiopathic AIHA, anti-Rs comprised IgG alone (IgG1 alone, IgG1+3 or IgG1+2+3 in each one). All of the 5 αMD induced anti-Rs comprised IgG1 alone. Observation of the course of AIHA revealed that, although IgG3 tended to correlate with their anemia, this trend was not universal. Besides, the G-sub were not related to the anti-Rs titers. Furthermore, the immunological aberrations in patients with AIHA+SLE were found to be more complicated.
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