Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
Volume 40, Issue 8
Displaying 1-11 of 11 articles from this issue
  • Hiroshi SAO, Kenjiro KITAORI, Chiaki KATO, Tatsuya ADACHI, Hiroaki YAM ...
    1999 Volume 40 Issue 8 Pages 630-638
    Published: 1999
    Released on J-STAGE: April 28, 2009
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    We studied the immune reconstitution and incidence of chronic graft-vs-host disease (GVHD) and infection in 25 patients who survived more than 1 year after bone marrow transplantation (BMT) from unrelated donors for treatment of leukemia.
    Of the 25 patients, 4 died after 1 year post-transplantation. The causes of death were relapse of leukemia (2 patients), obstructive bronchiolitis (1 patient), and hepatic failure (1 patient).
    Only 2 of 14 patients who were under 30 years of age at the time of their BMT developed chronic GVHD, however, 7 of 11 patients 30 years of age or older developed chronic GVHD. Lower karnofsky scores were displayed by 6 of the older patients with chronic GVHD but by none of the younger patients with chronic GVHD. These results underscored the importance of care for older patients with chronic GVHD. CD4-positive T cells, and especially CD4 * CD45RA-positive T cells, had not recovered to a normal range even 2 years after BMT. Longer follow-up will be necessary.
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  • Yoriko SHIMOMURA, Hisashi TSURUMI, Michio SAWADA, Toshiki YAMADA, Take ...
    1999 Volume 40 Issue 8 Pages 639-645
    Published: 1999
    Released on J-STAGE: April 28, 2009
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    The aim of this study was to assess the clinical significance of serum soluble interleukin-2 receptors (sIL-2R) in non-Hodgkin's lymphoma (NHL). Using a sandwich ELISA method, serum sIL-2R levels were measured in 720 samples from 87 patients with NHL (including 65 untreated patients) and 36 patients with other diseases such as infectious mononucleosis. The mean serum sIL-2R level in NHL was 4,017 U/ml (mean±SD, 4017±6352 U/ml). Patients in clinical stages III/IV (5116±6629) had significantly higher sIL-2R levels than those in clinical stages I/II (813±611). Patients with sIL-2R levels exceeding 8,000 U/ml had significantly lower survival rates (2-year survival: 12.3%) than those with sIL-2R levels below 8,000 U/ml (2-year survival: 76.0%) (P<0.01). Multivariate analysis of variables including age, clinical stage, LDH, CRP, performance status, number of extranodal diseases, and sIL-2R demonstrated that sIL-2R and LDH were significant prognostic indicators of overall survival. The upper limit of the 95% confidence interval for maximum sIL-2R level in follow-up of patients with complete remission was 2,014 U/ml. Although an increased sIL-2R level of around 2,000 U/ml in the remission stage did not necessarily suggest relapse of NHL, it did seem to warrant careful follow-up. The serum sIL-2R level appears to reflect tumor activity and may prove to be a useful prognostic indicator in patients with NHL.
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  • Toshio KATAYAMA, Hidekazu MASUOKA, Kaichi NISHIWAKI, Yohji OGASAWARA, ...
    1999 Volume 40 Issue 8 Pages 646-651
    Published: 1999
    Released on J-STAGE: April 28, 2009
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    A 60-year-old woman was admitted to our hospital in February 1993 due to dizziness, dyspnea, abdominal pain, and high susceptibility to bleeding. Physical examination revealed livedo reticularis of the foot, but did not detect hepatosplenomegaly. Examination of the peripheral blood detected pancytopenia, leukoerythroblastosis, and tear-drop erythrocytes. Primary myelofibrosis (PMF) was diagnosed on the basis of bone marrow biopsy findings. Antiphospholipid syndrome (APS) was confirmed by positive response to anti-cardiolipin antibody and recurrent splenic infarction. Because of factor XIII deficiency, the patient experienced severe gingival bleeding after tooth extraction. Her condition was complicated by mesenteric arterial thromboembolism and she died of sepsis 5 years after onset. Although the incidence of immunopathy in PMF patients is high, few studies to date have focused on APS patients presenting with a variety of severe embolic symptoms. Our patient required careful monitoring due to bleeding tendency and thromboemboli.
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  • Toshiyuki BOHGAKI, Atsushi NOTOYA, Masaya MUKAI, Michifumi KOHNO
    1999 Volume 40 Issue 8 Pages 652-657
    Published: 1999
    Released on J-STAGE: April 28, 2009
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    A 27-year-old pregnant woman was admitted to a local hospital because of headache, nausea, and general fatigue. Her blood examination showed leukocytosis, anemia, and thrombocytopenia. She was referred to our hospital in March 1998. Her bone marrow was normocellular with an excess of blasts (89.1%, peroxidase stain (-), PAS stain (-)) that displayed a positive immunophenotype for CD2, CD4, CD5, CD7, CD34, CD38, and CD71. Chromosome analysis revealed complex abnormal karyotypes. The patient was given a diagnosis of acute lymphoblastic leukemia associated with central nervous system and breast infiltration, and received induction chemotherapy during the second trimester of her pregnancy. After she achieved complete remission, a cesarean section was performed, and a healthy baby delivered. Our experience in this case demonstrated that combination chemotherapy during the second trimester of pregnancy is feasible.
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  • Hiroki YABE, Isaku SINZATO, Kimio HASHIMOTO
    1999 Volume 40 Issue 8 Pages 658-662
    Published: 1999
    Released on J-STAGE: April 28, 2009
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    A 15-year-old girl was admitted to our hospital because of fever, diarrhea, and right lower abdominal pain on November 11, 1997. Computed tomographic and ultrasound studies of the abdomen disclosed enlarged mesenteric lymph nodes. Hematologic and serologic findings included WBC 3000/μl, LDH 550 IU/l, IFN-γ 264 pg/ml, IL-6 9.74 pg/ml, sIL-2R 781 U/ml, and ferritin 720 ng/ml. Although the patient was treated with antibiotics, high fever and abdominal pain persisted with progressive anemia and leucocytopenia (1800/μl). Bone marrow aspiration specimens revealed an increase of histiocytes with phagocytosis. Appendectomy and lymphadenectomy were performed on November 21. A lymph node specimen showed necrosis with infiltration by large mononuclear cells. The resected appendix revealed reactive lymphoid hyperplasia. Based on these findings, a diagnosis of necrotizing lymphadenitis (NL) was made. The postoperative course was satisfactory and systemic symptoms resolved gradually without specific treatment. However, high fever and abdominal pain recurred with right cervicial lymph node swelling on December 15. The patient's general condition improved after treatment with prednisolone. In NL, lymphadenopathy is usually observed in the cervical region, and the involvement of intra-abdominal lymph nodes is quite rare. Our findings indicated the possibility that IFN-γ may play an important role in the pathogenesis of NL with hemophagocytic syndrome.
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  • Hisako OKURA, Masayuki HINO, Saori NISHIKI, Kenichiro KONO, Taro HASEG ...
    1999 Volume 40 Issue 8 Pages 663-666
    Published: 1999
    Released on J-STAGE: April 28, 2009
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    A 25-year-old woman was admitted to our hospital because of dark red urine in 1993. A diagnosis of hemolytic uremic syndrome (HUS) because of findings of hemolytic anemia with fragmented erythrocytes, thrombocytopenia, and renal dysfunction. The patient achieved remission with steroids and diuretics. In 1998 she caught a cold and happened to take the nonsteroidal anti-inflammatory drug, pranoprofen. Six hours later, she was rehospitalized because of dark red urine. Hemolytic anemia, fragmented erythrocytes, thrombocytopenia and renal dysfunction were observed again, also. A diagnosis of HUS was made. The patient was treated with steroid pulse therapy, infusion of fresh plasma, and plasma exchange transfusion. She recovered completely. In 1993 she had taker pranoprofen just prior to her first HUS episode. This was a recurrent case of HUS induced by pranoprofen.
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  • Kazuko KUDO, Masahumi ITO, Keizo HORIBE, Katsuhiko IWASE, Seiji KOJIMA
    1999 Volume 40 Issue 8 Pages 667-672
    Published: 1999
    Released on J-STAGE: April 28, 2009
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    An 8-month-old boy was admitted because of paleness. Laboratory studies disclosed microcytic and hypochromic anemia: red blood cell count 156×104l, hemoglobin 3.5 g/dl, mean cell volume 66 fl, and reticulocytes 0.5 ‰. Serum iron was 433 μg/dl and exocrine pancreatic dysfunction was not observed. Examination of bone marrow revealed prominent erythroid hyperplasia; 18% of the erythroblasts were distinct ringed sideroblasts. Electron microscopic studies found intramitochondrial iron deposits in the erythroblasts. The patient was given a diagnosis of sideroblastic anemia and responded to oral pyridoxine (50 mg/day) with an immediate increase of reticulocytes to 97 ‰, resulting in an improved hemoglobin concentration. He has maintained remission for more than 1 year following discontinuation of pyridoxine, which was administered for 2 months.
    Congenital sideroblastic anemia is relatively rare and mostly occurs in males, suggesting an X-linked recessive mode of inheritance. Recently, X-linked sideroblastic anemia has been shown to be caused by missense mutations in the δ-aminolevulinic acid synthase (ALAS) gene. A point mutation in exon 5 of the ALAS gene was found in this patient. Iron-deficiency anemia is the most common hematologic disease of infancy and childhood, resulting from lack of sufficient iron for synthesis of hemoglobin. It is therefore mandatory to differentiate sideroblastic anemia from iron-deficiency anemia and other common anemias.
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  • Yuko MATSUMOTO, Masaki MORI, Nobuyuki KANAI, Masaaki TAKATOKU, Tohru I ...
    1999 Volume 40 Issue 8 Pages 673-677
    Published: 1999
    Released on J-STAGE: April 28, 2009
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    A 20-year-old woman visited a nearby hospital because of sudden, severe, and unusual genital bleeding. She also exhibited severe anemia and thrombocytopenia. In transit to our hospital, the patient suddenly suffered cardiac arrest and died soon thereafter despite immediate blood transfusion and therapeutic intubation. Thrombotic thrombocytopenic purpura (TTP) was initially diagnosed at autopsy due to the observation of numerous fragmented erythrocytes in peripheral blood, evidence of hemolysis, and thrombotic microangiopathy in multiple organs. In addition, histopathologic and serologic findings disclosed an association with systemic lupus erythematosus (SLE). Test for anticardiolipin antibody was positive, and hemophagocytic findings were detected in lymph node specimens. Reports of TTP in association with SLE have been increasing in recent years. However, the mechanisms correlating these two illnesses have not been identified. We speculated that the rapid clinical course in this case was attributable to TTP that had been provoked by endothelial microangiopathy due to SLE, and moreover, the fact that the patient's general condition had been seriously complicated by excessive menstrual bleeding.
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  • Masatoshi KANNO, Shinobu NAKAMURA, Chika UOTANI, Yasushi TERASAKI, Tsu ...
    1999 Volume 40 Issue 8 Pages 678-684
    Published: 1999
    Released on J-STAGE: April 28, 2009
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    Most cases of primary pleural malignant lymphoma develop following chronic pyothorax. We report a case of primary pleural non-Hodgkin's lymphoma without chronic pyothorax. A 63-year-old woman was referred and admitted to our hospital with a right pleural effusion that was detected during a routine physical checkup. Her liver, spleen, and superficial lymph nodes were not palpable on physical examination. The massive right pleural effusion and a pleural mass were demonstrated on chest X-ray films and thoracic computed tomograms. Diffuse large B-cell non-Hodgkin's lymphoma was diagnosed by needle biopsy from the pleura, and the clinical stage was IE. Pleural effusion specimens contained no identifiable lymphoma cells, and examinations for Mycobacterium species were also negative. Human herpes virus 8 (HIV-8) DNA was detected in lymphocytes from the perepheral blood and pleural effusion. Epstein-Barr virus-encoded small RNAs and HHV-8 DNA were both negative in biopsied tissue from the pleural mass. Although a complete remission was achieved, the lymphoma relapsed about 8 months later. The patient is currently receiving salvage chemotherapy. Cases of primary pleural non-Hodgkin's lymphoma with massive pleural effusion that are not preceded by chronic pyothorax or Kaposi's sarcoma are very rare.
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