Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
Volume 34, Issue 9
Displaying 1-14 of 14 articles from this issue
  • Hiroyuki SHIMIZU, Hisaya NAKADATE, Takashi TAGA, Jiro UTSUMI, Kenichi ...
    1993 Volume 34 Issue 9 Pages 989-996
    Published: 1993
    Released on J-STAGE: April 25, 2009
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    The clinical characterisitics and treatment outcome in 40 children with acute promyelocytic leukemia (APL) treated at institutions participating in the Children's Cancer and Leukemia Study Group (CCLSG) were studied retrospectively. The median age at diagnosis was 8 years old. Bleeding diathesis was the predominant presenting symptom (90%), associated with laboratory findings of disseminated intravascular coagulation. Hepatomegaly, splenomegaly and lymphadenopathy were observed in 35%, 10%, and 15% of the cases, respectively. The median WBC count was 4.25×109/l. Anemia (hemoglobin<8g/dl) and thrombocytopenia (<30×109/l) were present in more than half of the patients. Cytogenetic studies demonstrated the characteristic 15; 17 translocation in about 90% of the patients analyzed. Induction therapy consisted of cytosine arabinoside and an anthracycline, with or without other agents. Twenty-nine patients (73%) achieved complete remission (CR) while early fatal hemorrhage was the predominant cause of induction failure. The survival rates continued to decrease (28% at 3 years, 24% at 5 years, and 7.9% at 10 years) due to late marrow relapses. Anthracycline cardiotoxicity was fatal in three patients in remission. These clinical features of childhood APL should be taken into account in the developement of new protocols.
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  • Rikio TAKEDA, Taiju UTSUGISAWA, Kazunori MURAI, Yasushi NARIGASAWA, Ya ...
    1993 Volume 34 Issue 9 Pages 997-1001
    Published: 1993
    Released on J-STAGE: April 25, 2009
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    Bone marrow findings at the onset of disease were analyzed in five patients with drug-induced agranulocytosis to detect simple indices for a determination of the indications G-CSF therapy. Two patients showed severe marrow hypoplasia, extremely low ME ratio and complete absence of myelocytes or more mature neutrophils in their bone marrow. In these cases, the periods for recovery to 500 or more paripheral neutrophils per microliter were 5 and 9 days in a G-CSF-treated patient and a non-treated patient, respectively. On the other hand, the bone marrow of other three patients revealed normal or slightly high cellularity, moderately low ME ratio and appearance of myelocytes and more mature neutrophils. In the latter cases, the periods for recovery to 500 or more peripheral neutrophils were 3 days in all cases, regardless of whether G-CSF was administered or not. These findings suggest that G-CSF should be adminstered to drug-induced agranulocytic patients with severe marrow hypoplasia, extremely low ME ratio and absence of marrow neutrophilic cells.
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  • Akio URABE, Hideaki MIZOGUCHI, Fumimaro TAKAKU, Tamotsu MIYAZAKI, Akir ...
    1993 Volume 34 Issue 9 Pages 1002-1010
    Published: 1993
    Released on J-STAGE: April 25, 2009
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    The safety and efficacy of recombinant human erythropoietin (epoetin α) were investigated in adult aplastic anemia patients whose hemoglobin (Hb) concentration was less than 10g/dl. Epoetin α was given subcutaneously every day at a dose of 3,000IU/body for two weeks, and the dosage was increased to 6,000IU, 12,000IU and 24,000IU every two weeks when the increment of Hb was insufficient. In cases in whom Hb concentration increased by more than 1g/dl or whose transfusion requirements reduced to less than 50%, treatment was judged to be effective. The whole rate of efficacy was 34.5% (10/29). Response to epoetin α treatment was better in patients whose symptoms were relatively mild. Mild cases responded to the treatment with 6,000IU/body/day, although a dosage of 24,000IU/body/day was required in moderate or severe cases. Neither serious adverse effect nor abnormal laboratory findings were observed. These results suggest that high dose subcutaneous epoetin α treatment is effective for the aplastic anemia in terms of increasing Hb concentration and reducing blood transfusions.
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  • Junji TOMIYAMA, Hideki KUDOH, Fujihiko SUZUKI, Mikio AOKI, Yamao ADACH ...
    1993 Volume 34 Issue 9 Pages 1011-1015
    Published: 1993
    Released on J-STAGE: April 25, 2009
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    A case of non-Hodgkin's T cell lymphoma (diffuse lymphoma, large cell type) associated with marked eosinophilia and pleurisy in a 57-year-old male is reported. The leukocyte count was 12.5×103l and eosinophil count was 53% and the absolute count of 6.6×103l. The patient's serum and pleural effusion fluid, containing abundant lymphoma cells, showed eosinophil colony stimulating factor (Eo-CSF) activity. Conditioned medium (CM) prepared from patient's T cells (T-CM) produced Eo-CSF and this was enhanced by interleukin-2 (IL-2) stimulation. We demonstrated that the patient's serum contained a significant amount of interleukin-5 (IL-5) and the patient's T-CM, particulary after IL-2 stimulation contained a significant amount of granulocyte-macrophage colony-stimulating factor (GM-CSF).
    These findings suggest that Eo-CSF produced by neoplastic T cells or normal T cells activated by tumor antigen stimulated the production of eosinophils in this patient and that both IL-5 and GM-CSF might play a role in Eo-CSF activity.
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  • Hideharu MIYABAYASHI, Kiyoshi KITANO, Masuo TERASHIMA, Hiroshi SAITO, ...
    1993 Volume 34 Issue 9 Pages 1016-1021
    Published: 1993
    Released on J-STAGE: April 25, 2009
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    A 58-year-old man was admitted in May 1988, because of high fever, skin, pigmentation and body weight loss. Abdominal ultrasonography and generalized computed tomography examinations showed swelling of general lymph nodes and bilateral adrenal glands, splenomegaly, and lesion in the liver. Serum cortisol, urinary 17-OHCS and 17-KS level were within the normal range, while the ACTH level was elevated (189.9 pg/ml). ACTH overload test showed a non-reactive pattern, leading to a diagnosis of partial Addison's disease. He was also diagnosed as non-Hodgkin lymphoma, diffuse, large cell type (B) by a biopsy of the left supraclavicular lymph node. After combination chemotherapy, swelling of the supraclavicular lymph node diminished, followed by normalization of ACTH level and improvement of symptoms. Abdominal lymphadenopathy, hepatosplenomegaly and swelling of adrenal grands also decreased in size. Although involvement of tumor cells in adrenal grands sometimes occurs in malignant lymphoma, it is reported that more than 90% destruction of adrenal gland tissue is necessary to develop Addison's disease. It was suggested that the involvement of many lymphoma cells in both adrenal glands resulted in the development of partial Addison's disease in this case.
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  • Yoko IKEDA, Kentaro YOSHINAGA, Seiko IKI, Yoshiaki OHBAYASHI, Akio URA ...
    1993 Volume 34 Issue 9 Pages 1022-1026
    Published: 1993
    Released on J-STAGE: April 25, 2009
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    A 32-year-old man visited Kanto Teishin Hospital complaining of general fatigue in May, 1992. He had been diagnosed as having paroxysmal nocturnal hemoglobinuria since 1980, because of brownish urine in the morning. He received blood transfusion in 1980. In 1983, he was treated with medication. There was no remarkable improvement, however, and he stopped coming to the hospital. When he was admitted to our hospital, hemolytic anemia and hemosiderinuria were noticed. Sucrose hemolysis test and acidified-serum lysis test (Ham test) were both positive. Positive rates of decay accelerating factor and CD59 were 38.8% (control 100%) and 45.4% (control 100%), respectively. His diagnosis was thus confirmed. Bone marrow was slightly hypocellular, and erythroid cells were relatively hyperplastic (M/E ratio 0.68). The oral administration of iron and oxymetholone was not effective for anemia. He was treated with daily subcutaneous administration of recombinant human erythropoietin (EPO, 3,000U/body/day). His hemoglobin level increased from 7.5g/dl to 12.0g/dl in 4 weeks, and general fatigue disappeared. Since he had concurrent chronic hepatitis C, alpha-interferon was also administered and his hemoglobin level is now controlled between 10 and 11g/dl. This case suggests that EPO can be useful for treating hemolytic anemia, even though erythroid cells in the bone marrow are hyperplastic.
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  • Kazunari YAMADA, Tsuyoshi NAKAMAKI, Akihiro YOKOYAMA, Ken-ichiro HINO, ...
    1993 Volume 34 Issue 9 Pages 1027-1032
    Published: 1993
    Released on J-STAGE: April 25, 2009
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    A 78-year-old man presented with marked thrombocytosis (126.4×104l), low neutrophil alkaline phosphatase (NAP) score and an abnormal karyotype of 46, XY, del(20)(q11q13) (18 of 20 cells), without obvious anemia or ringed sideroblasts in bone marrow. He received ranimustine (MCNU) with a diagnosis of essential thrombocythemia. After 2 years, he was admitted because of macrocytic anemia. The peripheral blood smear showed anisopoikilocytosis with a few nucleated red blood cells. Moderate thrombocytosis (71.7×104l) and a low NAP score were also observed. Bone marrow aspiration revealed erythroid hyperplasia with a significant increase in ringed sideroblasts (85% of erythroblasts). Cytogenetic studies showed the same abnormal karyotype 46, XY, del(20)(q11q13) in 100% of metaphase cells as those at initial diagnosis. A diagnosis of sideroblastic anemia preceded by essential thrombocythemia was made. No rearrangement or amplification of c-src was revealed. The observation of the same chromosome abnormality (20q-) in differnt phases of this patient's disease indicates that chronic myeloproliferative disorders and myelodysplastic syndrome may share some borderline or transitional cases with a similar pathogenesis.
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  • Shin MATSUDA, Yurie SAITOH, Hiroyuki KANBAYASHI, Tetsugoro TANAKA, Hid ...
    1993 Volume 34 Issue 9 Pages 1033-1038
    Published: 1993
    Released on J-STAGE: April 25, 2009
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    A 46-year-old woman with acute promyelocytic leukemia (APL) was treated with all-trans retinoic acid (ATRA) and chemotherapy according to the AML-92, M3 regimen of the Japan Adult Leukemia Study Group (JALSG). Between days 7 and 18 of therapy, she suffered chest discomfort, fever, cough, dyspnea and general fatigue. A chest roentogenogram showed bilateral interstitial infiltrates. Her leukocyte count began to increase rapidly to 6,400/μl on day 14. Marked hypoxia (PO2 35.9 mmHg) suggested occurrence of retinoic acid (RA) syndrome. She underwent endotracheal intubation and mechanical ventilation with administration of methyl-prednisolone (m-PSL) pulse therapy. Her symptoms promptly abated. Therapy with ATRA was continued and her leukocyte count reached 44,800/μl on day 19 of therapy. She achieved complete remission on day 48.
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  • —A Case Report and Review of the Literature—
    Yoshio HATAE, Yumi OHKOSHI, Michihiko UENO, Hisaya NAKADATE, Susumu II ...
    1993 Volume 34 Issue 9 Pages 1039-1043
    Published: 1993
    Released on J-STAGE: April 25, 2009
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    Primary myelofibrosis (PMF) is regarded as a chronic myeloproliferative disorder. It is characterized by marrow fibrosis, leukoerythroblastosis, tear drop erythrocytes and extramedullary hematopoiesis. Most patients are in their late 50s when first diagnosed. Pediatric PMF is said to be quite rare. Here describe a female infant with PMF. The patient was born on Aug. 7, 1991. The pregnancy and delivery were uneventful. Hepatomegaly was noted soon after birth. Combined blood counts showed polycythemia and leukocytosis. It was thought to be extramedullary hematopoiesis due to intrauterine infection. She was followed up in another hospital, but since her condition was unchanged she was admitted to our hospital for further medical examinations at age 7 months. On the peripheral blood smear, there were tear drop erythrocytes, normoblasts and early myeloid elements. Repeated bone marrow aspirations were dry taps. This case presented the classical fidings of fibrosis of the bone marrow on bone marrow biopsy. She is in good health without any therapy until now. A review of 7 cases of PMF, including our case, in Japanese children was made and discussed in comparison to adult cases.
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  • Akira HORIKOSHI, Shigemasa SAWADA, Masumi AISO, Masaaki KAWAMURA, Yosh ...
    1993 Volume 34 Issue 9 Pages 1044-1049
    Published: 1993
    Released on J-STAGE: April 25, 2009
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    Two cases of acute promyelocytic leukemia (APL) treated with all-trans retinoic acid (ATRA) developed fever, dyspnea and chest pain. A chest roentgenogram showed bilateral pleural effusion (case 1) and bilateral interstitial infiltration (case 2). The first case was a 50-year-old female in her first relapse, who was initially diagnosed as having pleuritis tuberculosa and was treated with anti-tuberculotic agents. Her symptoms continued for 44 days and complete remission was achieved 53 days after commencing ATRA therapy. The second case was a previously untreated 46-year-old male. His case had been diagnosed as adult respiratory distress syndrome and he had been treated with prednisolone. His symptoms rapidly improved and complete remission was achieved 38 days after the ATRA therapy. This was the first report of patients in Japan considered to have developed “retinoic acid syndrome (RAS)”. In our five APL cases treated with ATRA, the syndrome was not always accompanied by peripheral blood leukocytosis even though the two cases with RAS showed higher leukocyte counts than the other two cases without RAS and also had DIC. We should pay attention to the severe respiratory symptoms that develop in APL patients after ATRA treatment and immediate steroid therapy is required for such patients.
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  • Tadashi KANOH
    1993 Volume 34 Issue 9 Pages 1050-1052
    Published: 1993
    Released on J-STAGE: April 25, 2009
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    Although bullous amyloid lesions are very rare, the cutaneous lesions of this type can be a crucial manifestation of plasma cell dyscrasia. [Case Report] A 66-year-old man with a six-year history of multiple myeloma (IgG-λ, λ-type Bence Jones proteins) was admitted to the hospital because of hemorrhagic bullous lesions of the skin, chronic diarrhea and general malaise. A diagnosis of myeloma-associated amyloidosis with renal failure was made. One month later, he died as a consequence of progressive renal failure and systemic amyloidosis. A postmortem examination revealed myelomatous infiltrations (bone marrow and kidneys) and widespread amyloid deposits (skin, heart, lungs, kidneys, liver and intestine). The histologic examination of a bullous lesion showed amyloid deposits with formation of an intradermal blister.
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