臨床血液
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
30 巻, 7 号
選択された号の論文の28件中1~28を表示しています
臨床研究
  • —細胞内DNA量の有用性—
    片野 直之, 鶴沢 正仁, 川井 進, 藤本 孟男
    1989 年 30 巻 7 号 p. 943-950
    発行日: 1989年
    公開日: 2009/02/02
    ジャーナル 認証あり
    The cellular DNA content of marrow blasts were measured by flow cytometry in 250 children with acute lymphoblastic leukemia (ALL). Abonrmal DNA stemlines were detected in 51 cases (26%) of 196 children with untreated ALL and in 10 cases (18.5%) of 54 children with relapsed ALL.
    In untreated cases, the distribution of cellular DNA content for DNA aneuploidy showed that all except one hypodiploid case, had hyperdiploid DNA contents (D1>1.0). Children with hyperdiploid DNA stemline had significantly better prognosis than did those with diploid DNA stemline (D1=1.0) in both low-and high risk groups; risk assignment was based on an initial WBC count and age at diagnosis. The relative risk of failure to treatment for the hyperdiploid group was one third that of the diploid group in low-risk ALL and one fifth that of the diploid group in high-risk ALL.
    In relapsed ALL, there was no significant corelation between the cellular DNA contents and their prognosis after relapse. DNA aneuploidy was detected more frequently in late relapsed cases (40%) than in early relapsed cases (6.2%).
    These results show that cellular DNA content before treatment is an important prognostic factor in childhood ALL.
  • 田中 一郎, 吉岡 章, 嶋 緑倫, 藤原 利治, 寺田 茂紀, 中井 寛明, 宮田 茂樹, 沢本 好克, 神末 政樹, 福井 弘
    1989 年 30 巻 7 号 p. 951-957
    発行日: 1989年
    公開日: 2009/02/02
    ジャーナル 認証あり
    Properties of F. VIII/vWF in highly-purified factor VIII concentrates were examined using monoclonal antibodies. The F. VIII: C levels obtained with the chromogenic assay agreed with those obtained with the one-stage clotting method. The ratio between F. VIII: Ag and F. VIII: C in Hemofil M and Monoclate was 1.09 and 1.34, respectively. The F. VIII: Ag levels assayed by monoclonal ELISAs were the same as those assayed by polyclonal ELISA, except that those assayed by C 5-ELISA tended to be higher. The ratio between F. VIII: Ag and vWF: Ag in Hemofil M and Monoclate was 105 and 45, respectively. Both concentrates lacked the large multimers of vWF and showed the intensification of the satellite bands. SDS-PAGE patterns showed almost no contamination. Immunoblot analysis revealed that F. VIII in both concentrates could react with 6 kinds of monoclonal antibodies to F. VIII. These results suggest that the fundamental structure of F. VIII molecule for coagulant activity in both concentrates are preserved.
  • 杉田 完爾, 中澤 眞平, 森 泰二郎, 西野 和良, 安倍 隆, 木下 明俊, 鈴木 敏雄, 斎藤 みどり, 菊池 英之, 小佐野 満, ...
    1989 年 30 巻 7 号 p. 958-966
    発行日: 1989年
    公開日: 2009/02/02
    ジャーナル 認証あり
    The leukemic cells of 19 patients (pts) out of 180 children with acute leukemia expressed both CD 19 (B 4) and CD 13 (MY 7) antigens. These biphenotypic leukemias (BiL) were divided into 3 groups on the basis of clinical features, antigen profiles and karyotypes.
    GroupI pts (N=6) were infants under one year of age with high initial white blood cell (WBC) count over 200,000/μl. The blasts of this group did not express CD 10 (J 5) antigen. In 4 of these pts, the blasts initially did not express CD 13 antigen, however, they became strongly positive after short-term culture without stimulation. Cytogenetic analysis revealed a breakpoint at 11 q 23. Group II pts (N=6) were often school age and had a high WBC count over 100,000/μl and CD 10 positive blasts. The blasts of 4 pts did not express CD 13 antigen until short-term culture. Cytogenetic marker was Ph1 chromosome. Group III pts (N=7) were often preschool age and the WBC count was lower than that of other groups. The blasts expressed CD 10 antigen with normal karyotypes or various karyotype abnormalities. Prognosis of pts with BiL was more poor than that of CD 13- common ALL, and among 3 groups survival of Group I and II was significantly shorter than that of group III.
    This study suggests that childhood BiL represents heterogenous leukemias. It is important to distinguish BiL in childhood acute leukemia and further devide into the groups in order to establish an adequate therapy for prognostically poor BiL.
  • CCLSG-L 841とI 841の治療成績(Phase III Study)
    小泉 晶一, 佐々木 邦明, 鶴沢 正仁, 三間屋 純一, 二宮 恒夫, 高上 洋一, 内海 治郎, 谷田部 道夫, 武田 武夫, 関根 勇 ...
    1989 年 30 巻 7 号 p. 967-974
    発行日: 1989年
    公開日: 2009/02/02
    ジャーナル 認証あり
    From 1984 to 1987, 144 previously untreated children with low (LR) or intermediate-risk (IR) acute lymphoblastic leukemia (ALL) were entered in the protocol L 841 or I 841, respectively. The patients in the LR group were randomized to receive regimen A (L 841 A) or B (L 841 B) and the patients in the IR group were randomized to receive regimen B (I 841 B) or C (I 841 C). L 841 A consisted of vincristine (VCR)+prednisone (PDN) for the remission induction phase and 18 Gy cranial irradiation combined with intrathecal methotrexate (MTX) for the central nervous system (CNS) leukemia prophylaxis. The maintenance phase consisted of MTX iv alternating 5-day course of VCR+PDN+6-mercaptopurine (6MP) at 2 wk-interval. In L 841 B, I 841 B and I 841 C, asparaginase (ASP) was added as a third drug. Adriamycin (ADM) and high-dose MTX (100 mg/kg) were additionally employed in the intensive phase of I 841 C. Thirty-nine, 20, 25 and 49 eligible patients were entered in L 841 A, L 841 B, I 841 B and I 841 C, respectively. The event free survival rate in each regimen was 50.5%±13.7% (M±SE), 100%±6.0% (p<0.01), 72.7%±9.8% and 40.7%±13.2% (p<0.1) at 4 years, respectively. These data suggested that the three drug combination in the remission induction phase was much more effective than the two drug combination regimen in LR ALL. Further studies will be needed to improve the EFS rate of the patients with IR ALL.
  • 王 晋源, 堀田 知光, 村手 隆, 井上 千加子, 斎藤 英彦, 池田 靖, 小川 洋史, 斎藤 明
    1989 年 30 巻 7 号 p. 975-979
    発行日: 1989年
    公開日: 2009/02/02
    ジャーナル 認証あり
    We investigated the inhibitory effect of autologous sera on erythroid colony formation (CFU-E) of bone marrow cells from patients with chronic renal disease and the clinical effect of recombinant erythropoietin.
    Colonies formed in cultures using autologous serum (AS) decreased in 15 among 30 cases as compared with those using fetal calf serum (FCS). This inhibitory effect of autologous sera was diminished by treatment with activated charcoal in all these cases. The degree of hemoglobin increase after administration of recombinant erythropoietin appeared to correlate with the intensity of inhibitory activity of AS.
    These data indicate the clinical significance of the inhibitor (s) of erythropoiesis in uremic sera and suggest that the clinical effects of erythropoietin in this disease are further improved if the inhibitor (s) can be effectively removed.
  • 宇塚 善郎, 斎藤 淑子, 高橋 晴彦, 小松 真理, 伊藤 隆唯
    1989 年 30 巻 7 号 p. 980-987
    発行日: 1989年
    公開日: 2009/02/02
    ジャーナル 認証あり
    Neutropenic enterocolitis, also known as typhilitis or ileocecal syndrome, in leukemic patients undergoing chemotherapy has a high reported mortality. A recent increase in the incidence of neutropenic enterocolitis is associated with aggressive chemotherapy for acute leukemia.
    In this report, we report the incidence, diagnosis, and treatment of neutropenic enterocolitis during low-dose DCMP and high-dose DCMP regimen for 95 adults with acute nonlymphocytic leukemia, and review the literature pertaining appropriate medical and surgical management, and method of prevention. Finally we propose the favorable results of gut sterilization for the treatment and prevention of the disease.
  • 丸岡 啓一, 山中 正義, 三砂 將裕, 中田 浩一, 塚田 順一, 永田 一彦, 佐藤 忠嗣, 森 直樹, 織田 進, 千葉 省三, 江藤 ...
    1989 年 30 巻 7 号 p. 988-993
    発行日: 1989年
    公開日: 2009/02/02
    ジャーナル 認証あり
    We investigated serum acid and alkaline RNase activities in 16 cases of acute crisis of chronic myelocytic leukemia (CML), 21 cases of untreated CML, and 13 cases of treated CML, to clarify clinical significance of the determination of RNase activities in acute crisis of CML.
    We obtained results as follows; the ratio of acid to alkaline RNase activities (Ac/Al ratio) of chronic phase of CML was 1.64±0.47 (mean±SD) in the untreated cases, and was 1.32±0.16 in the treated cases. The Ac/Al ratio always indicated over 1.0 in the chronic phase of CML without any relationship to treatments. On the other hand, in the cases of acute crisis, the Ac/Al ratio was significantly lowered (0.94±0.22) as compared to the chronic phase of CML (p<0.001), and was similar to that of acute leukemia.
    The acid and alkaline RNase activities of the blast from the patients with acute crisis of CML showed remarkably lower value than those of leukemic cells from patients with chronic phase of CML. Therefore, it was Suggested that the return to normal range of Ac/Al ratio in acute crisis of CML depended on marked decrease of RNase activities of blasts.
    Thus, serial determinations of the enzyme activities are considered to be one of useful tools for prediction of acute crisis of CML.
  • —日本の9施設・過去13年間の治療成績の集計—
    高木 敏之, 大熨 泰亮, 林 恭一, 大竹 茂樹, 三比 和美, 沢田 海彦, 倉石 安庸, 近藤 誠, 鈴木 久三, 尾山 淳, 土岐 ...
    1989 年 30 巻 7 号 p. 994-998
    発行日: 1989年
    公開日: 2009/02/02
    ジャーナル 認証あり
    The clinical data of 53 patients with Stage III-IV Hodgkin's disease collected from 9 institutions in Japan were analyzed for the efficacy of chemotherapy. CR rate (78%) and five-year relapse-free survival (RFS) rate (61%) were higher in the patients treated with VEPA/CHOP regimen than those in patients with VEMP/BONP or MOPP/C-MOPP regimen, although the difference was not statistically significant because of the small number of the patients. As to 14 patients treated with CHOP regimen, CR rate was 87% and RFS curve trended toward plateau at 67% after 2 years and 3 months from the initiation of chemotherapy. Salvage therapy with adriamycin-based combination chemotherapy achieved CRs in 9 of 14 (64%) patients who had been treated with VEMP, BONP, modified MOPP or C-MOPP regimen. The CHOP regimen was effective in the treatment of III-IV Hodgkin's disease but an alternative multidrug chemotherapy with ADM, CPM, VCR, BLM, etoposide and procarbazine is recommended for achieving a higher CR rate and a better RFS. Prospective study is needed to establish the standard chemotherapy for Hodgkin's disease in Japan.
  • —再発の部位および時期と予後—
    清水 宏之, 佐々木 邦明, 高上 洋一, 太田 茂, 藤本 孟男
    1989 年 30 巻 7 号 p. 999-1004
    発行日: 1989年
    公開日: 2009/02/02
    ジャーナル 認証あり
    One hundred and forty patients out of 511 children with ALL who were entered in the study of the Children's Cancer & Leukemia Study Group from 1981 through March 1988 had relapsed by August 1988. The sites of relapse were BM (including concurrent CNS relapse) in 96 cases (70%), CNS in 36 cases (25%), and testicles in 8 cases (5%). A second complete remission was induced in 57 of the 75 patients (76%) with ALL in the first BM relapse. The projected disease-free survival (DFS) for those in the second BM remission was 26.0% at 2 years and the length of the first remission was correlated with the outcome. In the patients in early marrow relapse within 12 months after diagnosis, the probability of maintaining a second remission at 2 years was 6.7% compared to 29.8% in those in intermediate marrow relapse. All patients in late relapse after more than 48 months from diagnosis are surviving with no evidence of disease.
    The outcome of the patients with isolated extra-marrow relapse was so favorable that the probability of maintaining a second remission for those in CNS relapse was 41.4% at 3 years, and 57.1% in patients in testicular relapse.
    These data suggest that 20-30% of patients relapsing after more than 12 months from diagnosis may be salvaged, but those who relapsed during active therapy within the first 12 months after diagnosis, other types of treatment should by considered.
  • 安田 正之, 延永 正
    1989 年 30 巻 7 号 p. 1005-1009
    発行日: 1989年
    公開日: 2009/02/02
    ジャーナル 認証あり
    Serum antibodies to cytoskeletal systems were measured by indirect immunofluorescence using human skin fibroblasts and HEp2 cells as substrate. Healthy adults had IgM antibodies to vimentin and cytokeratin at 60 times serum dilution. IgG concentration did not have a correlation with IgG anti-cytoskeletal system antibodies and IgM concentration correlated with anti-vimentin and anti-cytokeratin antibodies.
    In patients with adult T-cell leukemia (ATL), IgG antibody titer against actin and vimentin was increased in spite of a decreased IgG concentration. IgM antibodies to vimentin was decreased in its titer together with a decreased IgM concentration. Antibody titer to HTLV-1, leukemic cell counts in peripheral blood and disease type, acute or chronic, did not have correlations with anti-cytoskeletal system antibodies. A third of the patients with ATL showed negative anti-EBNA antibody, suggesting that the functional impairment in EBV-specific killer cells was present.
    In contrast, the patients with infectious mononucleosis showed increased serum IgM concentration and IgM anti-vimentin antibody titer. It was suggested that the autoantibodies to cytoskeletal systems associated with viral infection were mainly composed of IgG in ATL and of IgM in infectious mononucleosis.
症例
  • 竹田 豊彦, 村野 浩太郎, 茆原 博志, 滝 正志, 渡辺 昭彦, 山田 兼雄, 杉田 完爾, 中沢 眞平
    1989 年 30 巻 7 号 p. 1010-1015
    発行日: 1989年
    公開日: 2009/02/02
    ジャーナル 認証あり
    We reported a case of TMD with transient increase of tetrasomy-21 cells in a phenotypically normal newborn.
    The patient was admitted to the St. Marianna University Hospital due to hepatosplenomegaly on the 7th days after birth. Hematological findings on admission revealed remarkable leukocytosis (168,300/μl) with 79% blasts. Immunological studies of the blasts showed a positive reaction for platelet associated antigens, KOR-P77, AN50, TP80 and a pan-T antigen, TP40. Cytochemically blasts were strongly positive for acid phosphatase, positive for αNAE and weakly positive for PAS. The platelet peroxidase reaction was observed in rough endoplasmic reticulm of blast cells.
    Both immunological and cytochemical findings suggested that the blasts were of megakaryocyte lineage. Chromosomal analysis of the blasts showed 48, XX, +21, +21 (21-tetrasomy).
    After chemotherapy with PSL and 6MP, bone marrow showed a complete remission. But we thought it was spontaneous remission because PSL and 6MP were not effective to acute megakaryocytic leukemia (AMKL).
    Bone marrow cells were karyotypically normal on the 67th day of life when abnormal blasts were not observed in the bone marrow.
  • 桝屋 正浩, 大野 敏之, 三輪 啓志, 星野 賢一朗, 穴沢 博, 池田 健, 川上 恵基, 北 堅吉, 白川 茂, 難波 紘二
    1989 年 30 巻 7 号 p. 1016-1020
    発行日: 1989年
    公開日: 2009/02/02
    ジャーナル 認証あり
    We report a 35-year-old male with leukemic change and gastric involvement of Burkitt's lymphoma. A monoclonal immunoglobulin (Ig), IgM-κ, was detected in the serum by means of immunoelectrophoresis. Immunophenotypical analysis showed that the neoplastic cells were CD20+, OKIal+, CD10-, CD21-, surface Ig+(M-κ), and cytoplasmic Ig-. The neoplastic cells did not secret Ig by using of protein A plaque forming cell assay. Active transcription of Ig hevy chain genes was not detected by cell dot analysis of the neoplastic cells. These findings support the possibility that the presence of the monoclonal Ig in the serum does not result from secretion of Ig from the neoplastic cells. The shedding of surface Ig from the neoplastic cells might result in the occurrence of monoclonal Ig in the serum.
  • 関根 隆夫, 高木 幹郎, 上村 泰弘, 森 美貴, 和田 英夫, 南 信行, 出口 克己, 白川 茂
    1989 年 30 巻 7 号 p. 1021-1026
    発行日: 1989年
    公開日: 2009/02/02
    ジャーナル 認証あり
    A 51-year-old women in menopause was admitted because of intermittent purpura. She was diagnosed as having cyclic thrombocytopenia. One platelet cycle lasted for about 22 days with platelet count fluctuating from 1.0×104l to 31.0×104l.
    The platelet-associated immunoglobulin was correlated with the platelet cycle.
    The rheumatic factor, antinuclear antibody, antimicrosome antibody, anti-thyroglobulin antibody and platelet binding immunoglobulin were all positive but did not fluctuate during the cycle.
    The platelet life-span was shortened.
    The mean platelet volume fluctunated during each cycle.
    The lipid emulsion test was prolonged.
    Symptoms of Sjögren syndrome and purpura appeared at the same time.
    It was strongly suggested that purpura was caused by the cyclical destruction of platelets by reticuloendothelial system.
  • 三崎 義堅, 大坂 顯通, 吉田 稔, 佐々木 龍平, 坂本 忍, 三浦 恭定, 加納 康彦, 小林 幸夫, 平井 久丸, 高久 史麿
    1989 年 30 巻 7 号 p. 1027-1031
    発行日: 1989年
    公開日: 2009/02/02
    ジャーナル 認証あり
    A 20-year-old man was admitted to our hospital because of fever and knee joint pain on March 20, 1986. Physical examination revealed generalized lymphadenopathy and hepatomegaly. White blood cell count was 32,800 μl with 74.4% blast cells. Bone marrow was hypercellular with 93.6% blast cells. Blast cells were weakly positive for acid phosphatase and PAS stainings but were negative for peroxidase, sudan black B and esterase stainings. Cell surface marker analysis of blast cells disclosed that they were positive for anti-HLA-DR, CD19, CD24, CD33 and CD38, but were negative for CD10 and CD20. Cytoplasmic immunoglobulin of blast cells was negative and TdT activity by immunofluorescent method was positive. Chromosomal analysis of bone marrow samples revealed normal karyotype. Therefore, this case was diagnosed as having acute lymphoblastic leukemia (L2) and achieved complete remission with LVP therapy consisting of 1-asparaginase, vincristine and prednisolone. Gene analysis of blast cells disclosed germ-line configuration of both the immunoglobulin heavy chain gene and T cell receptor beta chain gene. We speculated that the phenotype of leukemic cells might precede the genotype in some cases of acute leukemia.
  • 前田 剛, 足永 武, 菊入 国康, 石山 直志, 高倉 昌之
    1989 年 30 巻 7 号 p. 1032-1036
    発行日: 1989年
    公開日: 2009/02/02
    ジャーナル 認証あり
    A 32-year-old female was diagnosed as having Ph1-positive chronic myelocytic leukemia (CML) on March 6, 1985. She was intermittently treated with busulfan or 6-mercaptopurine. Her regimen was changed on February 27, 1987 to interferon-α (HLBI, Sumitomo) because of leukocytosis (46,200/μl) with basophilia (45%) and splenomegaly refractroy to conventional therapy. She was admitted to our hospital on November 27, 1987 because of blastic crisis.
    Cytogenetic analysis on peripheral cells was repeated six times during the treatment with HLBI. The sixth analysis was done on bone marrow cells as well. Nineteen to 22 metaphases were analyzed by the trypsin G-banding method after short-term culture. Cytogenetic analysis of peripheral cells revealed 46, XX, Ph1 in 9% of metaphases and 47, XX, Ph1, +8, i(17q) in 91% on March 2, 1987, and 47, XX, Ph1, +8, i(17q) in 95.2% of metaphases and 48, XX, Ph1, +8, i(17q), +19 in 4.8% on December 11, 1987. Karyotypes of bone marrow cells on December 11, 1987 were 48, XX, Ph1, +8, +8, 4(17q) in 73.7% of metaphases and 47, XX, Ph1, +8, i(17q) in 26.3%.
    It was speculated that abnormal clones might have developed in other sites than bone marrow.
  • 藤枝 広巳, 北条 均, 大屋敷 純子, 大屋敷 一馬, 外山 圭助, 馬場 百合子
    1989 年 30 巻 7 号 p. 1037-1041
    発行日: 1989年
    公開日: 2009/02/02
    ジャーナル 認証あり
    We report here a patient with acute lymphoblastic leukemia (ALL) in whom hypofibrinogenemia developed during chemotherapy. The patient was a 65-year-old female who was diagnosed as having common ALL, and she was treated with BHAC-DMPV (enocitabine: 160 mg, daunorubicine: 40 mg, 6-MP: 35 mg, prednisolone (PSL): 60 mg, and vincristine: 2 mg). Hypofibrinogenemia appeared promptly each chemotherapy, including PSL was given. To ascertain a correlation between hypofibrinogenemia and the drugs given in this patient, a trial administration of PSL was attempted during a complete remission state. The level of fibrinogen, in terms of the amount of antigen or coagulability, decreased during PSL treatment, although the levels of AT III, plasminogen, α2PI·Plm complex, and FDP did not change. Thus, it is difficult to speculate that PSL induced destruction of leukemia cells and release of protease from the cells resulting in fibrinolysis and hypofibrinogenemia in this case. These findings also suggest that the administration of only PSL could induce hypofibrinogenemia.
  • 島田 秀人, 開之内 万里, 塩村 惟彦, 小原 八郎
    1989 年 30 巻 7 号 p. 1042-1046
    発行日: 1989年
    公開日: 2009/02/02
    ジャーナル 認証あり
    An 80-year-old male was admitted because of dizziness and palpitation. Laboratory investigation revealed pancytopenia. A bone marrow aspirate showed a markedly hypocellular marrow with 41.6% blast cells. Peroxidase activity was negative and PAS reaction was block positive in the blast cells. Surface markers of these cells were positive for HLA-DR antigen and partially positive for CD 13 (MY7). Other markers, such as T, B or myeloid antigens were all negative. These blast cells were classified as L1 according to the FAB system but suggested essentially unclassifiable in cell differentiation.
    The pationt was treated successfully with vincristine and prednisolone and induced into complete remission althouh repeated marrow examination findings revealed hypocellular.
    As for the classification of hypoplastic leukemia, lymphoid or primitive “stem cell” leukemia also should be considered as other categories of acute leukemias and be treated according to each case.
  • 沖野 栄蔵, 森 千明, 山崎 省行, 豊田 貢一, 山田 燦, 館 慶三, 四家 正一郎, 神崎 暁郎, 池田 明代, 八幡 義人
    1989 年 30 巻 7 号 p. 1047-1051
    発行日: 1989年
    公開日: 2009/02/02
    ジャーナル 認証あり
    We have experienced a case of cytohemolytic hereditary elliptocytosis (HE) in a six-year-old boy. Metabolisms of the erythrocytic membrane were investigated on the members from his pedigree.
    The results were as follows;
    1) The presense or abscence of ovalocytic HE were studied in his pedigree.
    2) Failure in the process of spectrin dimer to tetramer conversion was found.
    3) Although abnormality existed in conversion of D to T by the patient's α-chain spectrin and normal β-chain spectrin, no abnormality was recognized when normal α-chain and the patient's β-chain were combined
    4) Decrease of the α-chain (80 kd) domain and appearance of abnormal (74 kd) spot were found by two dimensional peptide mapping of spectrin.
    5) In his pedigree, neither patients with hereditary pyropoikilocytosis (HPP) nor carrier states were recognized.
    In summary, this patient's pedigree was considered to be HE [SP α1/74].
    This case appears to be the first case in Japan and only few cases have been reported in the world literature.
  • 谷澤 昭彦, 薗村 貴子, 若園 吉裕, 秋山 祐一, 久保田 優, 三河 春樹
    1989 年 30 巻 7 号 p. 1052-1057
    発行日: 1989年
    公開日: 2009/02/02
    ジャーナル 認証あり
    A one-year-old boy diagnosed as refractory anemia with excess of blasts in transformation is reported. Hematological examination revealed anemia, thrombocytopenia and the presence of blasts in both peripheral blood (3.5%) and bone marrow (20.1%) specimens. Chromosomal analysis showed abnormal karyotype; 48, XY, +21, +marker, r (7). Analyses with cytochemical stainings, electronmicroscopy and monoclonal antibodies to cell surface markers could not define the lineage of blasts.
    Induction chemotherapy was started with VP-16 (230 mg/m2 x 5 days) as a single agent and complete remission was achieved. Thereafter, he had been treated for 11 months with the intensive chemotherapy which consisted of VP-16, cytosine arabinoside, daunorubicin, vincristine, vinblastine, 6-mercaptopurine, prednisolone, mitoxantrone and CNS prophylaxis. He has been in complete remission for 18 months. The usefulness of VP-16 to MDS in pediatric patients is documented.
  • 山田 道夫, 小時田 宏仂, 金沢 重俊, 金山 広海, 鈴木 知己, 小笠原 善郎, 加藤 政孝, 福田 喜安, 佐熊 勉
    1989 年 30 巻 7 号 p. 1058-1062
    発行日: 1989年
    公開日: 2009/02/02
    ジャーナル 認証あり
    A 40-year-old male complained of swelling of the right mandible. It was a hard bone tumor which was 70×50×45 mm in size without tenderness. In serum, the level of IgG was 5814 mg/dl, IgA 95 mg/dl and IgM 8 mg/dl with monoclonal increasing of gamma and lambda-chain. No Bence Jones protein was detected. In a bone marrow aspirate atypical plasma cells were 7.3% in nucleated cells in slightly hypocellular marrow. X-ray examination disclosed a spicule formation surrounding the osteolytic focus in the mandible.
    In addition, a osteolytic tumor without spicule formation was found in a rib. The histological examination of biopsy specimen obtained from the mandibular tumor revealed that atypical plasma cells infiltrated the fibrous tissue with new bone formation. Spicule formation in the bone lesion of multiple myeloma is unusal.
  • 井本 しおん, 中川 俊太郎, 伊藤 光宏
    1989 年 30 巻 7 号 p. 1063-1066
    発行日: 1989年
    公開日: 2009/02/02
    ジャーナル 認証あり
    We report a rare case of adult T cell leukemia/lymphoma (ATLL) in which cardiac invasion was clinically demonstrated and treated effectively.
    A 45-year-old female was admitted because of exertional dyspnea and cervical tumors. The leukocyte count was 19,100/μl with 20% of flower cells. HTLV-I antibody was positive. She was diagnosed as ATLL and treated with VEPA.
    She got remission for a short duration which was followed by relapse.
    OPEC was started as salvage therapy. In the course, extensive pericardial effusion was found in chest X-P. Pericardial puncture demonstrated ATLL cells and high titer of free IL-2 receptor (57,400U/ml) in the effusion. It was diagnosed as pericardial invasion of ATLL cells.
    Chemotherapy was started with new combination of drugs (cisplatin, mitoxanthrone, ifosfamide, and prednisolone). Concomitanly pericardial drainage was performed and the drugs were administered directly into the pericardial cavity. The clinical improvement was obtained and pericardial effusion did not appear thereafter.
    She died 4 months after the diagnosis of cardiac invasion. On autopsy myocardial invasion was identified. The pericardium widely adhered and effusion measured 42 ml.
  • 島村 隆二, 金子 武生, 森岡 英次, 渋谷 恒文, 石橋 大海, 仁保 喜之
    1989 年 30 巻 7 号 p. 1067-1073
    発行日: 1989年
    公開日: 2009/02/02
    ジャーナル 認証あり
    We treated 54-year-old Japanese man with a large cell type of Sézary syndorome. He had generalized erythrodermia, superficial lymphadenopathy, atypical lymphocytes in the peripheral blood, anti-HTLV-I antibody negativity and chromosomal abnormality. The patient was a hepatitis B virus carrier, and was complicated with hepatocellular carcinoma and monoclonal gammopathy of IgG, λ type. Sézary syndrome is a T cell malignancy, the clinical course of which is relatively mild and chronic; accordingly, this case showed no crisis under chemotherapy. However, the patient died due to rapid growth of the hepatoma. Although case reports of Sézary syndrome complicated with other malignancies are very few, the occurrence of malignancies is possible because of decreased immunological function in the patients. In this case, hepatitis B virus might participate in the hepatic oncogenesis under dysfunction of helper/inducer cells. In addition, the complication of monoclonal gammopathy was also interesting from the standpoint of the helper function of Sézary cells.
  • 彌重 博巳, 藤井 浩
    1989 年 30 巻 7 号 p. 1074-1078
    発行日: 1989年
    公開日: 2009/02/02
    ジャーナル 認証あり
    We reported a rare case of triple cancers with acute lymphoblastic leukemia (ALL) associated with disseminated intravascular coagulopathy (DIC) after the operations of colon canccer and primary lung cancer.
    A 78-year-old Japanese male, who had been operated upon for colon cancer (adenocarcinoma) on March 1981, metastatic brain tumor (adenocarcinoma) on December 1986, and primary lung cancer (squamous cell carcinoma) on Febrary 1987, was admitted to our hospital because of severe general malaise on December 6 1987. On admission, he had mild hepatosplenomegaly and hemorrhage diathesis such as purpura. Serum LDH increased to 2,515 mU/ml. The white blood cell count was 6,210/μl with 53% leukemia cells, and the platelet count was 12,000/μl. A bone marrow was infiltrated with 96.0% leukemia cells. The leukemia cells stained positively for PAS and negatively for peroxidase. Immunological examination of leukemia cells showed that HLA-DR, TdT, B1 and J5 were positive and cytoplasmic Igμ and surface Ig were negative, indicating common ALL. The coagulation studies revealed that the activated partial thromboplastin time was prolonged to 42.0 seconds, FDP increased to 79.9 μg/ml, and antithrombin-III decreased to 62%. Chromosome analysis showed a 48, XY, +2, +21q-, t(9;22) karyotype. He was diagnosed as having Ph1 positive ALL associated with DIC. He was treated with vindesine, prednisolone, L-asparaginase, and adriamycin and complete remission (CR) was achieved after two months. But on August 1988, 8 months after CR, ALL and brain tumor relapsed and he died of pneumonia on September 19 1988.
  • 緒方 清行, 二木 真琴, 猪口 孝一, 五味 聖二, 大木 一郎, 桑原 哲夫, 檀 和夫, 厨 信一郎, 野村 武夫, 篠原 多美子
    1989 年 30 巻 7 号 p. 1079-1083
    発行日: 1989年
    公開日: 2009/02/02
    ジャーナル 認証あり
    A case of secondary erythroleukemia treated with apparent success with androgen is reported. The patient is 63-year-old Japanese female. She had a history of multiple myeloma and had been treated with melphalan, vincristine and prednisolone. She developed erythroleukemia 88 months after the initiation of chemotherapy, while her myeloma was in complete remission. She was treated first with vitamin D3 with no beneficial effect and subsequently with 0.5 mg/kg of mepitiostane. A hematologic improvement began two months from the initiation of androgen therapy, and a complete remission of erythroleukemia was attained thereafter. A chromosomal abnormality of bone marrow cells, which was observed at the time of developing erythroleukemia, also disappeared after the treatment. She remained in good condition and hematologic remission under the androgen therapy at the latest follow-up, 1-year after the development of erythroleukemia. Androgen therapy may be considered as a useful treatment for secondary erythroleukemia.
  • 中島 理晴, 福永 秀行, 天野 昌彦, 福田 恒夫, 寮 隆吉
    1989 年 30 巻 7 号 p. 1084-1088
    発行日: 1989年
    公開日: 2009/02/02
    ジャーナル 認証あり
    A 62-year-old man was admitted to our hospital with exertional dyspnea. On admission, neither hepatosplenomegaly nor lymphadenopathy were noted. Laboratory data revealed anemia (Hb, 4.8 g/dl), leukopenia (2,800/μl) and a normal platelet count (21×104l), The immature blast cells in the peripheral blood were 15%, which increased to 32% during his clinical course. On cytochemical studies, the blast cells had no staining with peroxidase, α-naphtyl-butylate esterase and PAS, although acid phosphatase was positive. More than 58% of the blasts were identified as being of megakaryocytic lineage by platelet peroxidase and by tests with monoclonal GP IIb/IIIa antibody. Bone marrow biopsy disclosed marked fibrosis. However, the patient constantly had normal counts of platelets ranging from 21×104 to 63×104l.
    This case provides evidence that the megakaryocytic leukemias can be categorized into two types, which are characterized by either undifferentiated or differentiated megakaryocytic leukemia cells.
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