Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
Volume 30, Issue 11
Displaying 1-39 of 39 articles from this issue
  • —by Microcytofluorometry with DAPI Staining after Removal of Wright-Giemsa Staining—
    Yutaka KOBAYASHI, Masaru OZAWA, Naoyuki MARUO, Motoharu KONDO
    1989 Volume 30 Issue 11 Pages 1915-1922
    Published: 1989
    Released on J-STAGE: February 02, 2009
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    In the myelodysplastic syndrome (MDS) patients the ploidy distribution of megakaryocyte DNA was rarely reported. We applied DAPI (4', 6-diamidino-2-phenylindole) staining for measuring nuclear DNA content in megakaryocytes which have been morphologically identified on the Wright-Giemsa stained smear in 8 normal subjects and 12 MDS patients. Briefly, megakaryocytes morphologically examined on a Wright-Giemsa stained smear were photographed, and were located. We then removed the Wright-Giemsa stains by immersing it in 50% ethanol, 37°C for 1 hour and 100% methanol, 37°C for 1 hour. The DAPI staining was performed in DAPI solution (DAPI 0.01 mg/ml, pH 7.4 Tris-EDTA-2 Na buffer solution and 0.01 mol 2-mercaptoethylamine hydrochloride were mixed at the ratio of 0.5:98.5:1.0) for more than 30 min. The amount of nuclear DNA in the megakaryocyte previously identified was measured by cytofluorometry.
    The population of megakaryocyte in the normal subjects was the largest in the 16N, and in the 10 cases of the 12 MDS patients was the largest in the 8N, in the other 2 cases was the largest in the 4N. These results represent the development of the megakaryocyte nucleus in the MDS patients may be disturbed.
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  • Yuichi SATO, Ryosuke ITO, Toshimitsu SAITO, Yoshimasa SUTO, Noboru TAT ...
    1989 Volume 30 Issue 11 Pages 1923-1930
    Published: 1989
    Released on J-STAGE: February 02, 2009
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    Twenty children with acute lymphoblastic leukemia (ALL) in relapse were treated with various combinations of anti-leukemic agents in approach to the induction of remission. Among them, the four-drug reinduction regimen consisting of prednisolone, vincristine, daunomycin and L-asparaginase (PVDA regimen) was found to be highly effective. The incidence of remission was 100% (2/2) for first reinduction. The regimen also proved to be effective in the management of multiple subsequent relapses. The rate of remission induction for second and third to fifth reinduction attempts were 60% (3/5) and 50% (4/8), respectively. The over all remission rate was 60%. The median duration of subsequent remission for the entire patients treated by PVDA regimen was short, only 11 weeks (range: 3 to 26 weeks). PVDA regimen was effective in reinduction of remission in childhood ALL with relapse, but was not effective in remission maistenance. Optimal maintenance therapy for subsequent remissions has to be determined.
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  • —Interindividual and Intraindividual Variations—
    Nobuko HIJIYA, Yasuhisa KATO, Takeji MATSUSHITA, Tomomasa YOKOYAMA
    1989 Volume 30 Issue 11 Pages 1931-1936
    Published: 1989
    Released on J-STAGE: February 02, 2009
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    The pharmacokinetics of oral 6-mercaptopurine (6-MP) was assessed in ten children with acute lymphoblastic leukemia during maintenance chemotherapy. The doses were 175 mg/m2, 87.5 mg/m2, and 50 mg/m2. The relation between doses and the means of AUC (area under the curve) and Cmax (maximum concentration) suggested a non-linear pharmacokinetics. Our results demonstrated wide interindividual variability as reported by others. We demonstrated individual variation between clinically stable patients. The relationships between predicted and observed concentrations were variable among four patients studied. Some of the patients showed discrepancy between the both concentrations, wheras others did not. The results indicate that serum concentration after oral administration is predictable by further analysis of therapeutic drug monitoring.
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  • —120 Cases Analysis in Nagoya Bone Marrow Transplantation Group—
    Hironori YAMADA, Hideo TAKEYAMA, Akihiro ABE, Eiji WATANABE, Yoshihisa ...
    1989 Volume 30 Issue 11 Pages 1937-1942
    Published: 1989
    Released on J-STAGE: February 02, 2009
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    Results of the bone marrow transplantation (BMT) for 120 cases of leukemia, which were done in nine institutes in Nagoya (Nagoya Bone Marrow Transplantation Group) last ten years, were analyzed to determine the factors associated with an increased risk of developing interstitial pneumonia (IP). IP developed 49 out of 120 patients (49.8%) and case fatality rate was 63.3%. The median time from transplantation to onset of IP was 81 days (range 13-575 days), in 30 out of 49 cases (61.2%), this complication developed within 100 days after transplantation. Of the 49 patients who developed IP, cytomegalovirus (CMV) infection was associated in 18 cases (36.7%), no cases of P. carinii infection was detected. Five factors were associated with an increased risk for developing IP, (1) older age (≥10 years 47.0%: <10 y. 10.0%) (p<0.01) (2) disease stage at BMT (non-remission 76.2%: remission 32.5%) (p<0.01) (3) presence of acute GVHD ((+) 52.5% (-) 28.8%) (p<0.05) (4) onset day after BMT (≤100 days 61.2%: >100 d. 38.8%) (p<0.01) (5) sex matching between donor and patient (sex match 28.8%: sex mismatch 57.1%) (p<0.01). Three factors were mentioned in decreasing survival rate after developing IP, (1) disease stage at BMT (non-remission 6.3%: remission 57.7%) (p<0.01) (2) older age (≥30 years) (IP (+) 13.6%: IP (-) 66.7%) (p<0.01) (3) onset day of IP (≤100 day 16.7%: >100 d. 68.4%) (p<0.01), especially complicated with CMV infection. In recent five years, the rate of CMV-infected IP, of which fatality rate was known to be high, has decreased remarkably. Although, the progress of prophylaxis and treatment for CMV-infected IP, it has not been observed the improvement of incidence of IP. It must be discussed what is the cause of non-CMV idiopathic IP.
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  • Hideki OSAWA, Yoshihisa WAKABAYASHI, Shun-ichi HIROSE, Takeshi MIYANO
    1989 Volume 30 Issue 11 Pages 1943-1947
    Published: 1989
    Released on J-STAGE: February 02, 2009
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    Splenic autotransplantation after splenectomy for idiopatic thrombocytopenic purpura (ITP) is one of the effective treatments for maintaining the patients' immunity in children.
    We investigated this surgical method in 10 adult ITP patients. This method was extremely effective for thrombocytopenia. Cellular immunity of the patients was maintained as evidenced by the normal response of peripheral lymphocytes to phytohemagglutinin (PHA) and concanavalin-A (Con-A) after the operation. The mitogenic response of peripheral lymphocytes to PHA and Con-A might be a useful indication for predicting the effect of splenectomy.
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  • Y. TAKEMOTO, H. WADA, T. NSHIMURA, Y. TANAKA, T. AKAGI, S. ANDOH, K. O ...
    1989 Volume 30 Issue 11 Pages 1948-1953
    Published: 1989
    Released on J-STAGE: February 02, 2009
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    The outcome of chemotherapy in patients of 150 cases of acute leukemia was investigated. Patients were divided into two groups, that is group I: 100 cases of acute leukemia treated between 1980-1984, group II: 50 cases of acute leukemia between 1985∼1986. Complete remission was achieved in 66% of group I and in 82% of group II. Using multivariate analysis, the initial levels of FDP and WBC count and age were found to be important factors to induce complete remission. Female sex was suggestive of a longer remission duration and survival by discrimination analysis.
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  • Takeshi TOKIOKA, Yoshinori SHIMAMOTO, Jun NOMURA, Eriko FUJIWARA, Yumi ...
    1989 Volume 30 Issue 11 Pages 1954-1957
    Published: 1989
    Released on J-STAGE: February 02, 2009
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    A 57-year-old woman who suffered from acute myeloblastic leukemia during the course of chronic thyroiditis, is described. The patient was diagnosed as having chronic thyroiditis in 1984 when she was 53 year-old, and was treated with L-T4·Na. She admitted in July 1988 because of general fatigue, fever, cough and sore throat. On admission, hematological examination in the peripheral blood showed marked anemia and increased leukocytes with 20.5% leukemic cells positive for peroxidase staining. Bone marrow aspiration showed 38.8% leukemic cells. She was diagnosed acute myeloblastic leukemia. She reached complete remission after combination chemotherapy.
    The case of acute myeloblastic leukemia associated with chronic thyroiditis is rarely reported. We reviewed the literature and discussed acute myeloblastic leukemia associated with chronic thyroiditis including this case.
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  • Yoshio HATAE, Takeo TAKEDA, Hisaya NAKADATE, Yukiko HATAYAMA, Akira SA ...
    1989 Volume 30 Issue 11 Pages 1958-1962
    Published: 1989
    Released on J-STAGE: February 02, 2009
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    Two cases of malignant lymphoma in childhood were studied. The first case was a Japanese girl aged 8, in whom the primary site was skin of the right temple. The second case was a 4-year-old Japanese boy, who had metastases to the abdominal skin. Histochemical findings indicated B-cell lineage in both cases.
    Primary cutaneous lymphoma is extremely rare in childhood. Fourteen such cases that have been reported in Japan and our case added to them, were reviewed. The relationship between their morphologic, immunohistochemical and clinical findings were summarized and discussed. Although the prognosis of lymphoma confined to skin in childhood has been reported not to be bad as compared with other types of lymphoma, our first such case was fatal. This suggests that appropriate initial treatment is very important. Recent advances in science may clarify the clinical and biologic characteristics of this tumor in the near future.
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  • Kunihiko HISATAKE, Shizue WADA, Kouichiro IYOTA, Fumihiko TANIOKA, Mic ...
    1989 Volume 30 Issue 11 Pages 1963-1968
    Published: 1989
    Released on J-STAGE: February 02, 2009
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    A 37-year-old male was diagnosed as having chronic myelomonocytic leukemia (CMMoL) with chief complaint of systemic lymph node swelling. On admission, his peripheral blood revealed mild anemia and mild thrombocytopenia with giant plateletes, and monocytosis (1480/μl). NAP score was low. Serum lysozyme increased. The bone marrow showed normocellularity consisting of 4% myeloblasts and 14.4% promyelocytes, and a few myeloid cells were positive for double staining by α-naphthyl butyrate and naphthol ASD chloroacetate esterase. Biopsied specimens of the cervical lymph node showed infiltration of monocytoid cells, which were positive for lysozyme staining, into interfollicular tissue. As for chromosome variation, 21 large satellite was observed in all dividing cells from his bone marrow and peripheral blood. Furthermore, hemolytic anemia with hemoglobinuria developed during his course. Sugar water test was positive, but Ham test negative. Coombs test and Donath-Landsteiner reaction were negative. Abnormal hemoglobin, spherocyte and fragmentation were not found. Hemolysis disappeared about two months later. However, blastic crisis appeared and he died.
    We showed a case of CMMoL with 21 large satellite and paroxysmal nocturnal hemoglobinuria (PNH)-like complication. Satellite have usually been reported as asymptomatic, and thus this chromosome variant and CMMoL may have been coincidentally observed.
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  • Hiroki TAKAHASHI, Tatsumi NAKANO, Akihiro ONO, Toshiyuki HIGASHIDE, To ...
    1989 Volume 30 Issue 11 Pages 1969-1974
    Published: 1989
    Released on J-STAGE: February 02, 2009
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    A 65-year-old male was admitted to our hospital due to recurred malignant lymphoma of left tonsil origin. Studies using flow cytometry on mononuclear cells in peripheral blood revealed the appearance of intermediate B cells, and examinations on gastrointestinal tract showed diffuse infiltration of medium-sized lymphoid cells into stomach and colon, that had the same phenotype as tumor cells in peripheral blood. They suggested leukemic change and gastrointestinal tract infiltration of malignant lymphoma.
    Southern blot analysis revealed T-cell receptor β-chain gene rearrangement as well as immunoglobulin gene rearrangement. Analysis by histo in situ hybridization on infiltrated gastric mucosa specimen showed diffuse expression of immunoglobulin mRNA in tumor cells, but no message of T-cell receptor β-chain gene. Northern blot analysis showed the same result. It suggests that in this case, the rearrangement of T-cell receptor β-chain gene is ineffective rearrangement without transcription to mRNA.
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  • Hisashi SAKAMAKI, Satoko NAKAMURA, Yasuo MARUYAMA, Kaoru NAGATA, Yasus ...
    1989 Volume 30 Issue 11 Pages 1975-1981
    Published: 1989
    Released on J-STAGE: February 02, 2009
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    Ph1-positive chronic myelocytic leukemia (CML) developing in a treated case of acute promyelocytic leukemia (APL) is reported. The patient was a 62-year-old male who was diagnosed as having APL in December 1978. He was treated with daunorubicin, Ara-C and 6MP and a complete remission was obtained 4 months later. But APL recurred in February 1981. He was treated with BHAC, aclarubicin, 6MP and prednisolone. He remained in continuous complete remission for 5 years, when all therapy was discontinued. After then, the leukocytes count continued to rise and a diagnosis of Ph1-positive CML was made in September 1986. His leukocytes count has been well controlled with the use of busulfan. The event in this case suggests a possibility of the Ph1-positive CML being a secondary disease related to prior long term chemotherapy.
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  • Yasusada KAWADA, Midori SHIIKI, Takayuki MIYAGAWA, Yasuhiro KAJIWARA, ...
    1989 Volume 30 Issue 11 Pages 1982-1986
    Published: 1989
    Released on J-STAGE: February 02, 2009
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    A 2-month-old boy was admitted to our hospital because of poor sucking and jaundice. There were no abnormalities during the whole period of pregnancy and at birth. His mother was a HBeAb positive HBsAg carrier, but prophylactic maneuver such as anti-HB immunoglobulin and HB vaccine was not performed on him at birth. Physical examination on admission revealed mild disturbance of conciousness. The laboratory findings showed marked increments of serum bilirubin, GOT, GPT, and NH3, and prolongation of prothrombin time, activated partial thromboplastin time and hepaplastin test. Thus, he was diagnosed as fulminant hepatitis and treated with exchange tranfusion once or twice a day. Biochemical data improved gradually, but hypocoagulable states remained unchanged. At that time we decided to use Factor VII concentrate, because we found that, among several coagulation factors, factor VII activity decreased most rapidly after exchange transfusion. The alternate therapy of exchange transfusion and Factor VII concentrate improved his coagulation abnormality without any side effects.
    Our experience suggests that the combination therapy of exchange transfusion and Factor VII concentrate may be useful for management of fulminant hepatitis, particularly for uncontrollable coagulopathy.
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  • Shogo BANNO, Noboru HIRASHIMA, Toshiaki NODA, Masakazu NITTA
    1989 Volume 30 Issue 11 Pages 1987-1991
    Published: 1989
    Released on J-STAGE: February 02, 2009
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    We report a patient with acute nonlymphocytic leukemia (FAB classification: M 2) with trisomy 4, which is the first case in our country.
    A 42-year-old man was admitted to our hospital because of fever and general fatigue in May, 1988. His WBC count was 8,100/μl with 90% of leukemic cells and bone marrow smear showed 76.1% leukemic cells. The chromosomal analysis of marrow cells by G-banding revealed 47, XY, +4. In spite of administration of chemotherapy complete remission was not obtained, and he died of septic shock and severe liver damage 4 months after making the diagnosis.
    Chromosomal abnomality of trisomy 4 has been reported to be associated with predominantly either M 4 or M 2, and to be less than 0.1% of incidence in ANLL, according to the Second MIC Cooperative Study Group. It is suggested that trisomy 4 may be caused by exposure to some environmental factors such as toxic substances, since this chromosomal abnomality has been reported in the last 10 to 20 years.
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  • —A Case Report and A Review of Literature—
    Kazuya FUKUOKA, Kiyoshi NISHIKAWA, Yasuko MIZUMOTO, Taketo SHIMOYAMA, ...
    1989 Volume 30 Issue 11 Pages 1992-1996
    Published: 1989
    Released on J-STAGE: February 02, 2009
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    A 39-year-old woman was admitted to our hospital complaining of hemosputum and right neck swelling. Pharyngography, neck CTscan and laryngoscopy revealed moderately differentiated squamous cell carcinoma of the right pyriform sinus. After series of examinations, it was found that she had pancytopenia, hypoplastic bone marrow, hyperpigmentation of the skin, cardiac anomaly, small stature, hypogonadism, chromosomal aberrations and consanguinity in her parents. These findings suggested that she was the congenital aplastic anemia, that is Fanconi's anemia, variant form. Although pepleomycin and corticosteroids were administrated for the treatment of squamous cell carcinoma and aplastic anemia, she died of cardiovascular shock due to massive hemorrage. Flow cytometric analysis of squamous cell carcinoma showed an unusual aneuploidy DNA histogram.
    This is the first report on Fanconi's anemia with squamous cell carcinoma in Japan. It is said that chromosomal aberrations and impairment of DNA cross-links repair may play an important role developing of malignancy in Fanconi's anemia.
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  • Makoto FUTAKI, Toshiharu ITO, Koichi INOKUCHI, Takashi YAMADA, Ichiro ...
    1989 Volume 30 Issue 11 Pages 1997-2001
    Published: 1989
    Released on J-STAGE: February 02, 2009
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    A 26-year-old male was admitted to our hospital because of fever and leukocytosis. On admission, a white blood cell count was 28,300/μl with 46.5% blast cells and 16.0% atypycal monocytoid cells, a hemoglobin level 13.7 g/dl, and a platlet count 15.0×104l. Bone marrow contained 58.8% of peroxidase-negative blast cells. He was diagnosed as acute lymphoblastic leukemia (ALL L2) according to the FAB classification. Chromosome analysis revealed the marrow cells to contain 45, XY, -7, t(9;22) (q34;q11). On surface marker analysis, the leukemic cells were positive for both lymphoid (CD10) and myeloid markers (CD13). Two color flow-cytometric analysis showed two distinct populations with CD10 and CD13, respectively. Rearrangements of both immunoglobulin heavy chain and T cell receptor β-chain were observed. The “breakpoint cluster region” on chromosome 22 was not rearranged. On the basis of these findings, we thought this case being acute mixed leukemia.
    He was refractory to AdVP therapy and BHAC-DMP therapy. He is now under treatment with A-Triple-V therapy.
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  • Nobuhiro NOMURA, Hiroyuki UCHIDA, Hisashi ROPPONGI, Norihiko TONOZUKA, ...
    1989 Volume 30 Issue 11 Pages 2002-2007
    Published: 1989
    Released on J-STAGE: February 02, 2009
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    It is widely known that multiple myeloma is sometimes followed by amyloidosis. It is also not particularly rare that inclusions exist in myeloma cells. However, there has been no previous report of a case of myeloma with both inclusions and amyloidosis.
    A 60-year-old female initially complained of a tendency to bleed, which was caused by fibrinolysis. Amyloid deposition in bone marrow stroma and the gastric submucosa was recognized, in addition to crystalline inclusion in the cytoplasm of myeloma cells. An immunoelectron microscopic study demonstrated the amyloid fibrils and the crystals to react positively to anti-λ serum.
    No crystals were found in macrophages, and no relationship was recognized between lysosomes and crystalline inclusions in the cytoplasm of myeloma cells.
    This case had disturbed transportation or secretion of the λ type L chain and it was considered that crystals derived from the λ type L chain were formed in the cytoplasm of myeloma cells.
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  • Kazutoshi KOIKE, Takashi HANADA, Ichirou ONO, Youji NAGAI, Kenji SHIN, ...
    1989 Volume 30 Issue 11 Pages 2008-2013
    Published: 1989
    Released on J-STAGE: February 02, 2009
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    A 4-year-old girl was admitted because of fever, swelling of left chest wall and left axillary lymphadenopathy. Chest XP revealed left pleural effusion. Ga-scintigram showed multiple accumulation in skull, left ribs and iliac bone. A diagnosis of childhood Ki-1 lymphoma was made from the pathological findings of tumor in the skull. Immunopathological study revealed that the neoplastic cells were CD8 positive (suppressor phenotype).
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  • Junichi INOUE, Masahiro NOMURA, Emiiru AKAGI, Yoshito YAWATA
    1989 Volume 30 Issue 11 Pages 2014-2019
    Published: 1989
    Released on J-STAGE: February 02, 2009
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    A Japanese family of congenital high red cell membrane phosphatidylcholine hemolytic anemia (HPCHA) is reported. The propositus was a 48-year-old woman, who had been followed up as hemolytic anemia of unknown origin and undergone splenectomy. She showed no improvement after splenectomy. She also had primary biliary cirrhosis, of which a diagnosis was made based on laboratory data and liver biopsy. Red cell morphology demonstrated stomatocytosis with erythroid hyperplasia in the bone marrow. Abnormal hemoglobins and the red cell enzyme activities were not demonstrated. A marked abnormality was noted in red cell membrane lipids, specifically the elevation of phosphatidylcholine (PC) and free cholesterol (FC), despite of normal plasma lipids and lecithin cholesterol acyltransferase activity. Sodium transport, both influx and efflux, was increased. Therefore, the diagnosis of HPCHA was confirmed. The three additional cases were found in her pedigree. The data on the red cells of her mother and elder sister were similar to hers with respect to the red cell lipids and sodium transport. The propositus and her mother showed no improvement of anemia or icterus after splenectomy. There are only three reports of the family with HPCHA in the world.
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  • Tomonori SAWATARI, Fumio KAWANO, Yoshitaka AKAHOSHI, Norio ASOU, Kouji ...
    1989 Volume 30 Issue 11 Pages 2020-2023
    Published: 1989
    Released on J-STAGE: February 02, 2009
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    A 56-year-old man was admitted to the hospital in April, 1986, with the chief complaint of fatigue. The diagnosis of acute promyelocytic leukemia (APL) was made based on the proliferation of atypical promyelocytes in the bone marrow. No gingival swelling was found on admission. A complete remission was achieved by the BHAC-DMP therapy and maintained by the consolidation therapy and the intensification therapy. In July, 1987, he noticed a solitary gingival tumor around the left lower second molar. The biopsy showed the massive infiltration of leukemic cells, despite the hematological remission. Combination chemotherapy was not effective but the tumor disappeared by irradiation. A hematological relapse occured in November, 1987, but the second complete remission was achieved by the AB-triple V therapy. He died because of the second hematological relapse in July, 1988. Along with the wide use of intensive chemotherapy for acute leukemia, tumor forming leukemia during the hematological remission, as seen in this case, would be increasing. Thus, we should not overlook any newly formed tumor in the treatment of leukemia.
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  • Kunihiko ITO, Satoshi MURAKAMI, Nariaki OKU, Nobuhide TAKEDA, Naohisa ...
    1989 Volume 30 Issue 11 Pages 2024-2028
    Published: 1989
    Released on J-STAGE: February 02, 2009
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    A 31 year-old male who was treated with radiatoin under the diagnosis of malignant lymphoma was admitted to our hospital because of systemic erythema and tumor of bilateral upper arms in October, 1987.
    Leucocyte count of peripheral blood showed 4,400/μl with 36% leukemic cells and bone marrow was hypercellular with 85.6% leukemic cells. Leukemic cells were negative for peroxidase reaction and lineage specific monoclonal antibodies such as CD3, CD4, CD8, CD10, CD19 and CD20. T cell receptor (TCR) δ gene was rearranged but TCRβ, TCRγ and immunoglobulin (Ig) genes were in germline configuration.
    He was treated with combination regimen of doxorubicin, vindesine, prednisolone and L-asparaginase, and complete remission was obtained.
    These observations suggest that TCR δ gene rearrangement is useful for determination of clonality in cases without rearrangements of the other TCR and Ig genes.
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  • Satoshi HASHINO, Masahiro IMAMURA, Hajime KOBAYASHI, Masanori TANAKA, ...
    1989 Volume 30 Issue 11 Pages 2029-2034
    Published: 1989
    Released on J-STAGE: February 02, 2009
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    Two patients of acute myeloblastic leukemia (M2) with post-transfusional hepatitis (non-A, non-B) were treated with α-IFN and high-dose SNMC before allogeneic bone marrow transplantation. Bone marrow transplantation from HLA identical and MLR negative sibling donor was carried out when their hepatic functions were almost normalized. In the early phase after bone marrow transplantation, the hepatic function in both two cases has been stable, thus indicating that this treatment should be tried for reducing hepatic dysfunction and for safety bone marrow transplantation.
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  • Yutaka TAKAI, Hisatomo HAYASHI, Fumihiko ISHIMARU, Toshiaki FUJITA, Na ...
    1989 Volume 30 Issue 11 Pages 2035-2040
    Published: 1989
    Released on J-STAGE: February 02, 2009
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    Combined deficiency of factor V and factor VIII, a rare bleeding disorder, was found in a 43 yearold male. He had often presented manifestations of easy bruising since childhood, but none of his family had shown evidence of a bleeding tendency.
    We examined him and his family as far as we could and his abnormality of blood coagulation was apparent, but the members of his family were normal.
    The prothrombin time and activated partial thromboplastin time of this patient were prolonged, but his thrombin time was normal. Factor V and factor VIII coagulant activity were low, but von Willebrand factor antigen and activity (ristocetin cofactor activity) levels were normal.
    Protein C and Protein C inhibitor antigen and activity levels were also found to be normal. Following 1-deamino-8-D-arginine vasopressin (DDAVP) injection, he had immediate increases in factor VIII coagulant activity, but both von Willebrand factor antigen, activity levels and factor V coagulant activity remained low. Moreover, there was no rapid decline in factor VIII complex activity.
    These findings suggest that the endogenous factor VIII in this patient is metabolized normally and that at least the deficiency of factor VIII does not result from accelerated degradation in plasma.
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