臨床血液
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
23 巻, 8 号
選択された号の論文の13件中1~13を表示しています
臨床研究
  • 森山 美昭, 漆山 勝, 小山 覚, 広沢 秀夫, 藤原 正博, 高井 和江, 岸 賢治, 高橋 益広, 酒井 力, 眞田 雅好, 柴田 昭
    1982 年 23 巻 8 号 p. 1177-1182
    発行日: 1982年
    公開日: 2009/01/26
    ジャーナル 認証あり
    It is clinically important to settle the target point of chemotherapy for remission induction of acute leukemia. In this communication, we investigated the ralationship between leukemic cells left in marrow and the recovery of marrow granulocyte precursors (CFU-c) in remission and non-remission patients with acute leukemia after the cessation of chemotherapy.
    At the end of chemotherapy, % leukemic cell left in marrow was 0∼0.76% (mean: 2.03%) in the remission group and 1.8∼25.6% (mean: 9.46%) in the non-remission group. The recovery of marrow CFU-c appeared to be prompt in the remission group after the cessation of chemotherapy, but did not in the non-remission group. This CFU-c recovery and remission of acute leukemia did depend on leukemic cells left in marrow after chemotherapy, but not on the number of marrow nuclated cells left. These data suggest that leukemic cells left in marrow at the end of chemotherapy may affect the recovery of marrow CFU-c as well as remission induction of acute leukemia. Therefore, the end point of chemotherapy (target point) should be settle, at least, below 10% of blasts in marrow. This point seemed to be a rule for any chemotherapy used in acute leukemia.
  • 田口 博國, 新谷 憲治, 久保西 一郎, 三好 勇夫, 塩見 文俊, 町田 健一, 上田 尚紀
    1982 年 23 巻 8 号 p. 1183-1191
    発行日: 1982年
    公開日: 2009/01/26
    ジャーナル 認証あり
    A combination chemotherapy including adriamycin (ACOP and BACOP) was tried in 8 of 11 patients with adult T-cell leukemia. Relatively long survival time was achieved in 5 of these cases. Response to ACOP was transient in all the 8 cases and erradication of abnormal cells from the peripheral blood was not achieved. Repeated administration of ACOP was often necessary and addition of bleomycin was required to control rapidly increasing abnormal lymphocytes in the peripheral blood. The clinical courses of 3 selected cases are illustrated, whose survival time was 286, 257 and over 380 days. Hypercalcemia was observed in 2 of them and was treated successfully by the administration of ACOP and calcitonin. Herpes zoster was associated in all the 3 cases during the course of therapy. This complication was successfully treated with γ-globulin injection and rifampicin ointment. Trimethoprim-sulfamethoxazole was given in all the 8 cases for prophylaxis of Pneumocystis carinii pneumonia. Lethal complications were cryptococcus meningitis and pulmonary aspergillosis in 2 of them. Preventive measures against possible complications as well as effective chemotherapy must be sought in the treatment of ATL.
  • —化学療法による治癒の可能性—
    西原 龍司, 大熨 泰亮, 岡 瞭, 林 恭一, 佐藤 方春, 上岡 博, 安岡 正敏, 三道 康弘, 木村 郁郎
    1982 年 23 巻 8 号 p. 1192-1197
    発行日: 1982年
    公開日: 2009/01/26
    ジャーナル 認証あり
    The results of chemotherapy for non-Hodgkin's lymphoma (NHL) were analysed in 39 patients who had been resistered between January, 1971 and July 1978. Four-drug regimen consisting of bleomycin, vincristine, cyclophosphamide and prednisolone (BVCP or BCOP) was administered in 87% of the patients as an induction therapy. In patients with unfavorable histology (NH, DLPD, DM and DH), median survival of complete responders was significantly longer than that of partial responders and nonresponders (26 months vs 9 months in NH, DLPD and DM, 31 months vs 10 months in DH). Among 23 complete responders, 7 patients (5 DHs and 2 DLPDs) have been in complete remission for 18 to 64 months after cessation of all treatments. This stduy indicated that complete remission was essential for a prolongation of life of patients with NHL, and that advanced NHL was potentially curable by some relevant combination drug regiments.
  • 三上 定昭, 西野 正人, 安居 資司, 大久保 芳明, 嶋 裕子, 福井 弘
    1982 年 23 巻 8 号 p. 1198-1204
    発行日: 1982年
    公開日: 2009/01/26
    ジャーナル 認証あり
    Molecular size of factor VIII-related antigen (VIIIR: AG) were studied by SDS 1.5% polyacrylamide gel-crossed immunoelectrophoresis (SDS PAGE-CIE) on patients with various types of von Willebrand's disease (vWd) and their parents.
    1. Severe from vWd (3 families): VIIIR: AG in both parents of this form, carriers of heterozygote, showed low precipitin lines corresponding to 4×106-1×107 of molecular weight. It suggests that polymerizing function of VIIIR: AG exists normally in carrier, though productive function decreases to a half level.
    2. Classical form vWd (3 families): In this form, both a proband and one parent were the patients. Though precipitin lines of VIIIR: AG showed low arcs in all patients, they showed full polymerized patterns ranging 4×106 to 1×107 of molecular size. In regard to molecular weight of VIIIR: AG, there was no differenciation between a carrier of severe form and a patient with classical form.
    3. Variant form vWd-Subgroup A (2 families): VIIIR: AG in the proband of Family 7 showed the range of molecular weight between 1×106 to 3×106 daltons, showing 3 precipitin peaks. But VIIIR: AG in her mother (patient) ranged from 1×106 to 4×106 daltons, showing 4 peaks. Polymerizing ability of VIIIR: AG, therefore, was not on an appropriate level. On the other hand, the proband of Family 8 and his mother (patient) showed the same findings: precipitin lines with 5 peaks, molecular size of 1×106 to 5×106 and VIIIR: WF of 0.1 U/ml.
    4. Variant form vWd-Subgroup B (1 family): Molecular weight of VIIIR: AG in both proband and his mother showed a normal range of 1×106 to 1×107 dalthons. In this group, any defect of VIIIR: WF active site might be inherited dominantly.
症例
  • 伊藤 粋子, 広野 茂
    1982 年 23 巻 8 号 p. 1205-1210
    発行日: 1982年
    公開日: 2009/01/26
    ジャーナル 認証あり
    Hereditary hemorrhagic telangictasia (Osler-Weber-Rendu disease) is inherited as autosomal dominant disease. This disease is characterized by the presence of multiple telangiectasia of various organs which annoys patients with bleedings from various tissues. Two sibling cases with this disease were treated at our clinic because of long standing severe epistaxsis which never responded to several different therapies. Hemostatic examinations revealed no abnormality in both of them except weak Rumpel-Leede reaction in one of them. After oral administration of estrogen-progesterone, rapid improvement of epistaxsis was achieved in these patients. So far no successful report was published for the treatment of bleeding in this disease in Japan and our experience seems to suggest that administration of estrogen-progesterone is effective for this bleeding tendency.
  • 松本 孝夫, 杉本 正邦, 若林 芳久, 塩川 優一, 高久 史麿
    1982 年 23 巻 8 号 p. 1211-1216
    発行日: 1982年
    公開日: 2009/01/26
    ジャーナル 認証あり
    A 33 years old male was admitted to our hospital because of struma, palpitation and finger tremor. The diagnosis of hyperthyroidism was made by physical and laboratory examinations, then antithyroid drug (methimazole) therapy was begun. Twelve days after the start of the therapy, he showed high fever and leukocytopenia, especially neutropenia (350/cmm). Few days after withdrowing methimazole and begining of antibiotics, clinical and hematological findings were recovered normally.
    The effect of methimazole, serum and peripheral blood lymphocytes of patient on colony formation from CFU-C was examined in the semisolid agar culture system. There was substantial decrease in CFU-C colony numbers only when both methimazole and peripheral blood lymphocytes of patient were added in the culture. On the otherhand, neither methimazole only nor combination of methimazole and patient's serum suppressed the colony formation.
    It was suggested that peripheral blood lymphocytes were playing an important role in this suppression of CFU-C colony formation, and furthermore in the cause of agranulocytosis in vivo through the immunological mechanism.
  • 大川 正人, 外岡 立人, 松本 隆任, 上野 範博, 佐藤 佳子, 富安 孝文, 武田 武夫
    1982 年 23 巻 8 号 p. 1217-1221
    発行日: 1982年
    公開日: 2009/01/26
    ジャーナル 認証あり
    We report here a case of congenital acute lymphocytic leukemia (ALL) with a karyotype of 46, XY, t(4; 11) (q13; q23), which is thought to be the first case in Japan. It is well known that ALL frequently associates with chromosomal abnormalities, and that one of causative factor of congenital leukemia seems to be cytogenetic abnormalities. Thus we report the clinical feature and course of our case.
    The patient was two month-old boy, who was admitted to our hospital with the chief complaints of abdominal distension and tarry stool. Physical examination showed anemia and massive hepatosplenomegaly. Laboratory examinations on admission revealed remarkable leukocytosis (587,000/mm3) with 98% of lymphoblasts in peripheral blood in addition to anemia and thrombocytopenia. Blastic cells were PAS positive, and morphologically L1 type ALL was suggested. Chromosomal study on peripheral blood cells showed that lymphoblasts had an abnormal karyotype of 46, XY, t(4; 11) (q13; q23). The patient reached a complete remission by the induction therapy for 6 weeks consisted of VCR (1 x/W x6)' daunomycin (3 x/W x2) and prednisolone. He was further given L-asparaginase for 10 days as the consolidation therapy and cranial irradiations of 800 rads as the prophylaxis of meningeal leukemia, however, a hematological relapse occurred after one month in spite of the maintenance therapy with 6MP&MTX. As reported recently, these results of chemotherapy confirm that ALL with chromosomal abnormality of t(4q-; 11q+) was resistent to chemotherapy, and that protocols of chemotherapy for ALL may be not able to succeed in the maintenance of remission for long period in such cases.
  • —造血幹細胞の動態—
    三澤 眞人, 白 桂枝, 伊福 秀貴, 大江 与喜子, 岡本 隆弘, 藤田 茂, 野口 光二, 金丸 昭久, 永井 清保, 甲斐 俊朗, 原 ...
    1982 年 23 巻 8 号 p. 1222-1227
    発行日: 1982年
    公開日: 2009/01/26
    ジャーナル 認証あり
    A case of dyskeratosis congenita (Zinsser-Cole-Engman syndrome) with pancytopenia is pressented.
    A 32-year-old man was admitted to our hospital because of pancytopenia and pigmentary changes in the skin. Physical examination revealed leukoplakia on the tongue, nail atrophy and reticular pigmentation of the skin, resulting in the diagnosis of dyskeratosis congenita.
    In an attempt to clarify the cause of pancytopenia, the levels of the hemopoietic stem cells were estimated by clonal cell culture technique using methyl cellulose. The result showed the decrease in number of precursor cells in both the bone marrow and peripheral blood. The number of colonies from CFU-Mix, BFU-E, CFU-E and CFU-C per 2×105 bone marrow cells were 0 (2.3±1.3), 1.3 (65.7±27.3), 6 (183.2±84.3) and 13 (42.4±24.7), respectively (normal values in parentheses). No colony was detected for those in peripheral blood. Therefore, the pancytopenia was thought to be due to a defect at the level of multipotent stem cells.
    Frequent complications of malignancy have been reported in this unique disorder. As indicated in the reports, cancer of the tongue was found in this patient 6 month after diagnosis.
  • 副田 敦裕, 横井 茂夫, 千葉 博胤, 木谷 信行, 赤塚 順一, 仁科 甫啓
    1982 年 23 巻 8 号 p. 1228-1234
    発行日: 1982年
    公開日: 2009/01/26
    ジャーナル 認証あり
    Herediatary hemolytic anemia associated with glucosephosphate isomerase (GPI) deficiency was originally reported in 1968 by Baughan et al. Recently, we experienced the tenth patient with this disorder in Japan. The patient was a nine years old boy and refered to our hospital with complaints of fever, vomiting and general malaise. Physical examination revealed slight splenomegaly and functional heart murmur, but otherwise was within normal range.
    Laboratory studies showed he had nonspherocytic hemolytic anemia with Hb 6.4 g/dl, RBC 2.57×106/μL and Ht 24%. Autohemolysis test demonstrated Dacie “Type I”. Erythrocyte enzyme assay showed the red cell GPI activity of the patient and his mother were 59% and 48% of the nomal level, respectively. Micaelis constants and electrophoretic pattern of the GPI were normal. Thermostability of this enzyme was, however, normal. The findings mentioned above suggested that this patient was a new variant of GPI deficiency, as these paticular characterstics of the enzyme of this patient was distinct from the cases reported previously.
  • 三浦 琢磨, 田中 久夫, 吉成 道夫, 徳永 昭子, 古藤 しのぶ, 斉藤 和雄, 泉 二郎, 稲垣 稔
    1982 年 23 巻 8 号 p. 1235-1240
    発行日: 1982年
    公開日: 2009/01/26
    ジャーナル 認証あり
    It is said that scurvy is now an uncommon disease in the pediatric field. Only a few papers on this disease were reported in these five years.
    In our hospital a 5-month-old female baby was admitted with bleeding tendencies such as purpura, vomiting and bulging of the anterior fontanelle. On admission coagulation studies including bleeding time, PT, APTT, platelet aggregation (ADP, collagen, epinephrine and ristocetin) and so on, revealed no abnormal findings except positive Rumpel-Leede test. Roentgenograms of the lower extremities showed subperiosteal hemorrhage, thinning of cortex and a scurvy line. Subdural hematoma was found in CT-scanning and she was diagnosed as scurvy with subdural hematoma. We performed an operation of a subdural-peritoneal shunt and prescribed vitamin C. The prognosis was good.
    Since it was found that the formula milk for this baby had been prepared with boiling water, the level of vitamin C was assayed. The result revealed that the level of vitamin C in the formula with boiling water was decreased to 42.6% of the original source.
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