Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
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Volume 35, Issue 8
Displaying 1-14 of 14 articles from this issue
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  • Bone Marrow Transplantation From Donors Other Than HLA Matched Siblings for Hematological Malignancies
    Kimikazu MATSUMOTO, Keizo HORIBE, Yoshiki AKATSUKA, Saburo MINAMI, Tak ...
    1994 Volume 35 Issue 8 Pages 729-737
    Published: 1994
    Released on J-STAGE: April 25, 2009
    DOIhttps://doi.org/10.11406/rinketsu.35.729
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    One hundred and fourteen patients with hematological malignancies received bone marrow transplantation from donors other than HLA-identical siblings. Sixty-three patients received transplantations from related donors; 20 were phenotypically identical for HLA-A, B, D/DR (RM0), 32 differed at one locus (RM1) and 11 differed at more than one loci (RM2). Fifty-one transplantations were from unrelated donors; 37 were phenotypically identical and mixed lymphocyte culture (MLC) negative (UR0) and 14 were MLC positive (UR1). One hundred and four patients had durable engraftment. Four (RM1(1), RM2(2), UR0(1)) failed to achieve engraftment. In terms of the probability of ≥ Grade II acute graft-versus-host disease (GVHD), there was no significant difference among the groups according to HLA disparity (RM0: 25%, UR0: 33%, UR1: 39%, RM1: 47%, and RM2: 50%). The probability of chronic GVHD was significantly higher in UR0 and UR1 than RM0 (71%, 75% vs 28%, p<0.05). The disease-free survival at 3 years was 45% (RM0), 50% (RM1) and 42% (UR0). More than 50% of patients other than RM0 died of fatal complications including GVHD within 60 days after grafting. In conclusion, unrelated donor and related donor mismatched at one locus could be selected for marrow graft in the case of the absence of an HLA-matched related donor. However, more advances in post-transplant management and in histocompatibility testing should be required.
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  • Coordination of Unrelated Bone Marrow Donors in the Tokai Bone Marrow Bank
    —Psychological Reactions of Donors to Donation—
    Makoto YAZAKI, Yasuo MORISHIMA, Yoshihisa KODERA, Tatsuya YAMAUCHI, Hi ...
    1994 Volume 35 Issue 8 Pages 738-743
    Published: 1994
    Released on J-STAGE: April 25, 2009
    DOIhttps://doi.org/10.11406/rinketsu.35.738
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    The Tokai Bone Marrow Bank was established in 1989 and coordinated 1,415 patients with 3,000 HLA-A, B-typed donors. Of the 1,415 patients, 757 patients had HLA-A, B-identical donors, 206 patients had HLA-DR-identical donors, and 80 patients had MLC-compatible donors. Finally, 55 unrelated donor bone marrow transplantations were done. The most frequent reason of interruption in coordination was disagreement of donor's family. We sented several questions by mail around 1 month after the donation to 55 donors to analyze the psychological reactions of unrelated bone marrow donors to donation. Donors were generaliy quite positive about the donation. 92% of the donors felt it was worthwhile and no one felt it was not at all worthwhile. 73% of the donors would be willing to donate again in the furture. 79% of donors would like to know the clinical course of the patient after the bone marrow transplantation. We hope that these results may be helpful in the development of the Japan Marrow Donor Program.
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  • The Clinical Significance of Glycosylated Ferritin in Iron Overloads and Hematopoietic Malignancies
    Yoshio TAKAKUWA, Keisuke MIYAZAWA, Osamu YOSHIKAWA, Keisuke TOYAMA
    1994 Volume 35 Issue 8 Pages 744-750
    Published: 1994
    Released on J-STAGE: April 25, 2009
    DOIhttps://doi.org/10.11406/rinketsu.35.744
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    Serum ferritin concentration has been known to represent the amount of total body iron and has been clinically used as a parameter to evaluate the iron storage pool in a whole body. Glycosylated (secreted) and non-glycosylated (non-secreted) forms of serum ferritin (sFt) have been reported by others based on the difference in their affinity to concanavalin-A binding. In this report, we assessed the amount of glycosylated serum ferritin (Glyco-sFt) and the ratio (%) of Glyco-sFt/ in hematopoietic disorders including iron-overloads (n=10), leukemias (n=36), malignant lymphomas (n=10), multiple myelomas (n=3) and myelodysplastic syndromes (n=12). A high percentage of Glyco-sFt was observed in normal healthy controls (n=18, 78.1±7.4%) and iron-overloads (61.1±17.8%) as compared with that in hematopoietic malignancies (43.8±23.4%, p<0.001). The amount of Glyco-sFt in iron-overloads was higher than that in hematopoietic malignancies with hyperferritinemia (p<0.005). These data demonstrated that the same part of serum ferritin in hematopoietic malignancies was the non-secreted form and appeared to be derived from tumor cell lysis. We conclude that assessment of Glyco-Ft is a useful parameter to distinguish iron-overloads from malignant hematopoietic disorders both displaying hyperferritinemia.
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  • Cyclic Thrombocytopenia Associated with Erythroid Hypoplasia—A case—
    Naotoshi SHIBAHARA, Hakumei OH, Yasuhide SAITOH, Shigeaki SATOH, Masao ...
    1994 Volume 35 Issue 8 Pages 751-755
    Published: 1994
    Released on J-STAGE: April 25, 2009
    DOIhttps://doi.org/10.11406/rinketsu.35.751
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    We report the case of a 75-year-old woman with cyclic thrombocytopenia associated with erythroid hypoplasia. One platelet cycle lasted for about 28-30 days, with the platelet count fluctuating from 1.0×104/μl to 56.0×104/μl. Megakaryocyte count increased in the phase during which platelet count increased, and decreased in the phase during which platelet count decreased. Bone marrow colony formation was observed in serum-free agar, and megakaryocyte colony count was correlated with the platelet cycle. Platelet-associated immunoglobulin was in the normal range when platelet count increased, but increased when platelet count decreased. These findings suggest that the observed platelet count fluctuation was related to the production and destruction of platelet. Our patient also had erythroid hypoplasia, but her erythrocyte count did not fluctuate. This is the first reported case of cyclic thrombocytopenia and erythroid hypoplasia.
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  • Primary Plasma Cell Leukemia Complicated with High-output Cardiac Failure and Hyperammonemia
    Eita NAKAHASHI, Noriomi KURIBAYASHI, Norihide TSUJI, Tatsuya KAWAGUCHI ...
    1994 Volume 35 Issue 8 Pages 756-760
    Published: 1994
    Released on J-STAGE: April 25, 2009
    DOIhttps://doi.org/10.11406/rinketsu.35.756
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    A 23-year-old male patient with plasma cell leukemia showed characteristic clinical features: accelerating heart failure and consciousness disturbance accompanied with an increase of plasma cells in peripheral blood. Evaluation of cardiac function revealed a hyperdynamic cardiac state with low somatic vascular resistance, indicating high-output cardiac failure. However no disorders causing high-output cardiac failure were found. Consciousness disturbance and hyperammonemia with serum amino acid abnormality of unknown origin were also demonstrated. After intensive combined chemotherapy (MVD+VAD), high-output cardiac failure and hyperammonemia improved with disappearance of plasma cells, suggesting that these symptoms were closely related with progression of plasma cell leukemia.
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  • B-lymphoma Arising in the Temporal Muscle
    Rumiko KAGEYAMA, Teruyuki TAKASHIMA, Masafumi TANIWAKI, Shigeo HORIIKE ...
    1994 Volume 35 Issue 8 Pages 761-767
    Published: 1994
    Released on J-STAGE: April 25, 2009
    DOIhttps://doi.org/10.11406/rinketsu.35.761
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    B cell lymphoma arising in skeletal muscle is described with the review of literature. A 72-year-old man visited our hospital on September 21st 1992, because of a right temporal mass which had grown gradually since one year previously. A CT scan and MRI showed a soft tissue mass adjacent to the temporal muscle expanding from the right temporal to infratemporal fossa. Physical examination on admission revealed that the mass at the right temporal region, was non-tender, elastic soft, and 5×2 cm in diameter. Some elastic hard masses at the right infraauricular region, approximately 1.5×1.2cm in diameter, were also found. Histological examination of a biopsied specimen obtained from the temporal mass revealed B cell lymphoma, diffuse medium-sized cell type. Tests for surface markers using tumor cell suspension showed positive results for CD5, CD19, CD20, SmIg μ, δ, λ, but negative for CD10. Chromosomal analysis revealed clonal aberrations, and the defining karyotype as 51, X, +X, -Y, +2, +3, +4, +8, +12. The patient achieved a complete remission after treatment with combination chemotherapy including doxorubicin, cyclophosphamide, vincristine, etoposide, vindesine, procarbazine, and prednisolone.
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  • Autoimmune Hemolytic Anemia Associated with Multicentric Castleman's Disease with a 28-year History
    Jun-ichi HISATAKE, Taijiro ISHIYAMA, Yoshihisa AKIMOTO, Isao MATSUDA, ...
    1994 Volume 35 Issue 8 Pages 768-773
    Published: 1994
    Released on J-STAGE: April 25, 2009
    DOIhttps://doi.org/10.11406/rinketsu.35.768
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    A 49-year-old female admitted because of anemia. had skin rashes since age 20. Generalized lymphadenopathy and fever appeared and the patient was diagnosed as multicentric Castleman's disease (MCD) at 40 years of age. Lymphadenopathy and fever improved with combined chemotherapy. In November, 1992, anemia increased with reticulocytosis (11.8%) and laboratory examination revealed a positive result for Coombs test and increased indirect bilirubin. A diagnosis of autoimmune hemolytic anemia (AIHA) was made. Steroid and plasmapheresis showed temporary effects, but anemia relapsed when stevoids weae decreased. Immunosuppressive drugs, vincristine and danazole were ineffective. Anemia improved on the second attempt at steroid therapy. The level of Hb rose to 11.2g/dl after 3 months. The relationship between MCD and AIHA was discussed.
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  • A Patient with Adult T Cell Leukemia in Smoldering Stage Expressing an Aberrant Phenotype of CD3- and CD4+
    Kouji NASU, Hideyuki MATSUBAYASHI, Yoshihisa MAEDA, Ryoichiro MATSUURA ...
    1994 Volume 35 Issue 8 Pages 774-779
    Published: 1994
    Released on J-STAGE: April 25, 2009
    DOIhttps://doi.org/10.11406/rinketsu.35.774
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    This report is of a patient with adult T cell leukemia (ATL) in the smoldering stage showing expression an aberrant phenotype of CD3-, CD4+ and CD8-. A 71-year-old woman was admitted to our hospital in December 1992, because of skin eruption and persistent low grade fever. Laboratory examination showed a leukocyte count of 7,000/μl with 29% abnormal lymphocytes. The diagnosis of ATL was made by the detection of serum anti-HTLV-I antibody and the monoclonal integration of HTLV-I proviral DNA in abnormal lymphocytes. ATL cells at diagnosis were CD3+, CD4+ and CD8+. However, one month later ATL cells lacked CD3 and CD8. Three color analysis showed that most of the CD25+ cells were CD3- and CD4+.
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  • Pure Red Cell Aplasia with Thymoma, Myasthenia Gravis and Normal Pressure Hydrocephalus
    Yoshihisa AKIMOTO, Taijiro ISHIYAMA, Hideyuki UENO, Ken-ichiro HINO, S ...
    1994 Volume 35 Issue 8 Pages 780-785
    Published: 1994
    Released on J-STAGE: April 25, 2009
    DOIhttps://doi.org/10.11406/rinketsu.35.780
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    Cases of pure red cell aplasia with thymoma and myasthenia gravis are rare. We described a patient who had concomitant pure red cell aplasia, thymoma, myasthenia gravis and a normal pressure hydrocephalus. A 63-year-old man with disturbances of gait, left blepharoptosis and anemia was presented to our hospital. Laboratory examination on admission revealed severe anemia. Bone marrow aspirates showed erythroid hypoplasia of marked degree. Chest x-ray and chest CT revealed a tumor to the right of cardiac wall. The tensilon chloride test and antiacetylcholine receptor antibody were positive. A hydrocephalus was demonstrated with brain CT. Cerebrospinal fluid pressure was in the normal range. He was disgnosed as having pure red cell aplasia with thymoma, myasthenia gravis and a normal pressure hydrocephalus. This appears to be a fairly rare case. It seems important to consider that a normal pressure hydrocephalus may have immunological disorders.
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  • A Case of IgG-κ Type Multiple Myeloma Complicated by Fanconi Syndrome
    Shinji SAKAUE, Keizou NAKATA, Hiroshi NAGATA, Jun YAMAGUCHI
    1994 Volume 35 Issue 8 Pages 786-791
    Published: 1994
    Released on J-STAGE: April 25, 2009
    DOIhttps://doi.org/10.11406/rinketsu.35.786
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    Fanconi syndrome is characterized by some absorptive defects of proximal renal tubules. It is observed etther primarily, or secondarily to various diseases. A case of multiple myeloma complicated by Fanconi syndrome is reported. The patient was a 62-year-old female. The serum total protein was elevated, and monoclonal IgG-κ protein was found on serum immunoelectrophoresis. Urinary Bence Jones protein was positive. Bone marrow aspiration disclosed increased dysplastic plasma cells, which led to the diagnosis of multiple myeloma. A diagnosis of Fanconi syndrome was based on renal glycosuria, hypophosphatemia, hypokalemia, and panaminoaciduria due to abnormal excretion from the kidney, and metabolic acidosis. After chemotherapy for multiple myeloma, serum IgG level and urine sugar decreased, and serum potassium level was corrected.
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  • Multiple Myeloma with Numb Chin Syndrome as the Initial Manifestation
    Noriyuki SHIROSHITA, Mitsutoshi KUROSAWA, Mihiro OKABE, Makot CHUUMA, ...
    1994 Volume 35 Issue 8 Pages 792-797
    Published: 1994
    Released on J-STAGE: April 25, 2009
    DOIhttps://doi.org/10.11406/rinketsu.35.792
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    The numb chin syndrome (NCS) is characterized by chin or lower lip numbness restricted to the distribution of the mental nerve (the distal trigeminal nerve). A case of multiple myeloma with polycythemia vera was diagnosed with NCS as the initial manifestation. A 73-year-old Japanese male was admitted to our hospital in April, 1993, because of paresthesia around the chin and lower lip. X-ray film showed multiple osteolytic lesions. According to serum and urine immunoelectrophoresis, lamda type Bence Jones protein was demonstrated. The bone marrow aspiration showed the normocellular marrow with 14.1% myeloma cells. He was diagnosed a suffening from multiple myeloma and was treated with melphalan and prednisolone. He is alive at present with resolution of NCS. We discussed pathogenesis, diagnosis, and treatment of NCS.
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