Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
Volume 45, Issue 3
Displaying 1-13 of 13 articles from this issue
  • Yoshihiro YAKUSHIJIN, Ikuya SAKAI, Kiyonori TAKADA, Masaki YASUKAWA, S ...
    2004 Volume 45 Issue 3 Pages 218-222
    Published: 2004
    Released on J-STAGE: July 28, 2009
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    We encountered a case of a 59-year-old female who simultaneously contracted a non-Hodgkin lymphoma (NHL) and a plasma cell neoplasm. The patient consulted her physician about her abdominal tumor and anemia in March 1999. She was diagnosed as having NHL (follicular center lymphoma, grade I, stage IIA) after an open tumor biopsy, and treated by cycles of CHOP chemotherapy which resulted in complete remission. However, the patient's abdominal tumor appeared again in March 2000 and she was hospitalized at the Ehime University Hospital. A tumor biopsy was performed laparoscopically at that time. Follicular lymphoma (with positive LCA, L-26, and bcl-2 immuno-staining) with the development of retroperitoneal fibrosis was diagnosed again. When a bone marrow puncture was performed because of a condition of monoclonal gammopathy which had continued for two years, a smoldering myeloma was additionally diagnosed. This diagnosis was made after the presence of IgG-lambda M protein when the marrow showed an increase in the number of plasma cells. In a Southern blot analysis which studied the abdominal tumor and the bone marrow cells, each B-cell tumor had a different IgH gene rearrangement pattern. Therefore, this case was diagnosed as an example of the simultaneous existence of two different B-cell tumors. Double cancers in hematological malignancies are very rare and this was thought to be an interesting case.
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  • Michio SAKAI, Midori SHIMA, Yoichiro IZUMI, Akira SHIRAHATA
    2004 Volume 45 Issue 3 Pages 223-227
    Published: 2004
    Released on J-STAGE: July 28, 2009
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    A 17-year-old female underwent examination due to the chief complaints of epistaxis and bleeding from the gums. In addition to hemolytic anemia (Coombs positivity), she was found to have a marked reduction in the von Willebrand factor antigen (vWF:Ag) and ristocetin cofactor activity (vWF:RCo). Anti vWF antibodies mainly comprised of IgG1 fractions were also detected, so acquired von Willebrand syndrome (AvWS) with autoimmune hemolytic anemia (AIHA) was diagnosed. The recovery of vWF: Ag and vWF: RCo following administration of a factor VIII concentrate containing vWF was good, but the half life was short at about two hours and the collagen binding activity and vWF: RCo in mixing tests with control plasma were only mildly inhibited. Therefore, the anti vWF antibody in the present case was thought not to inhibit vWF function but to be involved in clearance promotion. Hemostatic control during hemorrhage was performed using a factor VIII concentrate containing vWF. With the administration of prednisolone thereafter, anti vWF antibodies disappeared at the same time as there was improvement in the AIHA. Throughout the course the patient contracted acute hepatitis A, became pregnant and gave birth, but there were no clear effects on the clinical phenotype of AvWS.
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  • Nobuhide KUSABA, Hiroshi YOSHIDA, Fumihiko OHKUBO, Kaori MISHIMA, Kazu ...
    2004 Volume 45 Issue 3 Pages 228-232
    Published: 2004
    Released on J-STAGE: July 28, 2009
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    A 68-year-old man was diagnosed as having bronchial asthma in November 1996. He presented with leukocytosis in June 2002. The WBC count was 29,900/μl with 82% mature neutrophils showing toxic granules. The neutrophil alkaline phosphatase score and serum level of vitamin B12 were elevated. Bone marrow demonstrated myeloid hyperplasia and plasmacytosis. Cytogenetic and molecular analyses were negative for Philadelphia chromosome and BCR/ABL fusion gene. Lambdatype Bence-Jones protein was detected on the serum and urinary immunoelectrophoresis. The coexistence of chronic neutrophilic leukemia and myeloma was suspected based on the clinical features. The serum level of granulocyte-colony stimulating factor (G-CSF) was elevated. Immunohistochemically, atypical plasma cells were positive for anti G-CSF antibody. Finally, we diagnosed this patient as having a G-CSF-producing myeloma. Treatment with melphalan and prednisolone was initiated without beneficial response. He was then admitted to our hospital for ROAD therapy (ranimustine, vincristine, melphalan, and dexamethasone). The neutrophil count decreased in parallel with the serum G-CSF level. These observations indicated that the neutrophilia in this case was probably caused by a reactive response to G-CSF secreted from the myeloma cells.
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  • Tomonobu SATO, Masahide NAKAJIMA, Akihiro IGUCHI, Ryoji KOBAYASHI, Kun ...
    2004 Volume 45 Issue 3 Pages 233-237
    Published: 2004
    Released on J-STAGE: July 28, 2009
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    We encountered a 12-year-old girl with acute promyelocytic leukemia (APL) that occurred 21 months after a living donor partial orthotopic liver transplantation from her father for ornithine transcarbamylase deficiency. FK-506 had been administered for prophylaxis against graft-versus-host reaction. The bone marrow specimen revealed a massive infiltration of promyelocytic blasts (M3 by FAB classification) with chromosome 46, XX, t(15;17)(q22;q12), being the recipient origin. A PML/RARα chimeric gene was detected by RT-PCR. The patient was diagnosed as having APL and successfully induced to complete remission by chemotherapy including daunorubicin (DNR), cytarabine (araC), and all-trans retinoic acid (ATRA). She has been in continuous remission for 12 months after the treatment. Leukemia after liver transplantation is generally taken as a rare complication. However, recent advances in the survival rate of patients who have undergone liver transplantation will lead to an increase of such cases.
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  • Takeshi INUKAI, Kanji SUGITA, Miwa GOTO, Makoto NAKAMURA, Toru TEZUKA, ...
    2004 Volume 45 Issue 3 Pages 238-242
    Published: 2004
    Released on J-STAGE: July 28, 2009
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    Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic disorder, caused by impaired cell surface expression of GPI-anchors in hematopoietic cells as a result of somatic mutation in the PIG-A gene, and often progresses into bone marrow aplasia. We experienced a girl diagnosed as having PNH with spontaneous recovery from pancytopenia, and analyzed the GPI-anchor expression on peripheral blood cells. Thrombocytopenia was first determined when she was 5-years old, and Ham and sugar water tests were negative at the age of 6 years. Subsequently, the pancytopenia slowly progressed, and the diagnosis of PNH was made at the age of 8 years based on the positive Ham test. Frame-shift of the PIG-A gene in exon 2 was confirmed in the peripheral granulocytes at the age of 11 years. Pancytopenia spontaneously subsided over two years after diagnosis, and an almost normal hematogram except for mild thrombocytopenia has been maintained for the subsequent 4 years. In periodical flow cytometric analysis of the CD55 expression on granulocytes and erythrocytes and the CD48 expression on lymphocytes, the population of the PNH clone was almost unchanged during the first two years of spontaneous recovery, but that of CD55-negative erythrocytes gradually decreased, suggesting that regression of PNH clone might be unnecessary in transient improvement of pancytopenia.
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  • Naoko WATANABE, Minako TANI, Yasuhiro TANAKA, Masayuki KURATA, Akiko M ...
    2004 Volume 45 Issue 3 Pages 243-246
    Published: 2004
    Released on J-STAGE: July 28, 2009
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    A 79-year-old man was admitted because of consciousness disturbance on August 9, 2002. He had been diagnosed as having chronic myeloid leukemia in 1999, and since then, he had continued to take hydroxyurea (1500 mg/day) orally. On admission, his serum sodium concentration was as low as 119 mEq/L, while urinary sodium excretion was high. Based on the blood picture and lack of hepatosplenomegaly, we considered that the leukemia was still in the chronic phase. Because of normal blood level of the antidiuretic hormone (ADH) concentration and sufficient urine volume, the syndrome of inappropriate ADH secretion (SIADH) was unlikely, and sodium-losing nephropathy was suspected. After discontinuation of hydroxyurea, the urinary sodium excretion decreased and the patient's consciousness became clear concomitantly with improvement in the serum Na level. This patient appears to be the first case of hyponatremia caused by hydroxyurea.
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  • Yoshihisa YAMAMOTO, Kazuyoshi ISHII, Shosaku NOMURA, Shirou FUKUHARA
    2004 Volume 45 Issue 3 Pages 247-249
    Published: 2004
    Released on J-STAGE: July 28, 2009
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    We report a 49-year-old woman who had adult T-cell leukemia/lymphoma (ATL) of the bone with multiple osteolytic lesions and pathological fractures of her extremities. The WBC count was 15,810/μl with 0-1% of abnormal lymphocytes, but CT scan revealed no lymphadenopathy. Antibodies to HTLV-I were positive, and a right tibial bone biopsy showed infiltration of ATL cells. Monoclonal integration of HTLV-I proviral DNA was demonstrated in bone tumor cells. Seven weeks later, she died of DIC due to severe infection during extensive chemotherapy.
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  • Yutaka TAKEI, Noriko USUI, Nobuaki DOBASHI, Takeshi HAGINO, Yutaka OKA ...
    2004 Volume 45 Issue 3 Pages 250-251
    Published: 2004
    Released on J-STAGE: July 28, 2009
    JOURNAL RESTRICTED ACCESS
    A 29-year-old man with diffuse large B-cell lymphoma treated by radiotherapy for his relapsed lesion followed by 4 doses of weekly rituximab was admitted to our hospital with interstitial pneumonia. After steroid pulse therapy, he had contraction of the left visual field. He was diagnosed as having progressive outer retinal necrosis due to a varicella-zoster virus that was detected from the left vitreous sample. Systemic antiviral treatment failed to prevent rapid development of whole layer necrosis of the left retina. Vitrectomy with silicone oil tamponade saved his final visual acuity. This unusual event might be related to rituximab causing deterioration of humoral immunity.
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