Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
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Volume 54, Issue 11
Displaying 1-13 of 13 articles from this issue
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Picture in Clinical Hematology
The 73th Annual Meeting of the Japanese Society of Hematology
Special Award Received Lecture
Review
Case Reports
  • Hematologic improvement with deferasirox following tandem antithymocyte globulin treatment in a transfusion-dependent patient with severe aplastic anemia
    Wataru JOMEN, Hiroyuki KURODA, Michiko YAMADA, Teppei MATSUNO, Masanor ...
    2013 Volume 54 Issue 11 Pages 2047-2052
    Published: 2013
    Released on J-STAGE: December 04, 2013
    DOIhttps://doi.org/10.11406/rinketsu.54.2047
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    A 62-year-old man with transfusion-dependent severe aplastic anemia received immunosuppressive therapy (IST) with rabbit antithymocyte globulin and cyclosporine A in April 2010. However, his transfusion dependency did not improve. As more than 100 red blood cell (RBC) transfusions had been performed, he was administered iron chelation therapy (ICT) with deferasirox (DFX) to improve iron overload starting in July 2011. Consequently, both RBC and platelet transfusion dependency gradually improved concomitant with a decrease in serum ferritin. The bone marrow (BM) biopsy findings before administration of DFX showed severe iron accumulation and strong positive immunostaining for 8-OHdG, a marker of oxidative stress due to free iron. One year after ICT, the number of BM hematopoietic cells was increased and both iron deposition and oxidative stress were decreased. These findings suggest that DFX may contribute to hematological improvement in patients with IST-refractory aplastic anemia.
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  • Successful treatment with rituximab in a patient with refractory mixed-type autoimmune hemolytic anemia
    Kaoru ONO, Tsutomu SATO, Satoshi IYAMA, Ayumi TATEKOSHI, Akari HASHIMO ...
    2013 Volume 54 Issue 11 Pages 2053-2055
    Published: 2013
    Released on J-STAGE: December 04, 2013
    DOIhttps://doi.org/10.11406/rinketsu.54.2053
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    The evidence that rituximab is effective therapy for refractory warm or cold autoimmune hemolytic anemia (AIHA) has been accumulating; however, the efficacy of rituximab for mixed-type AIHA is not evident. Herein, we report a case of mixed-type AIHA refractory to corticosteroids and splenectomy, but successfully treated with rituximab (375 mg/m2/day, once weekly, four times). She achieved a complete response, which has been maintained for 16 months, to date, despite steroid tapering. Our case suggests that rituximab therapy should be considered for refractory AIHA even of mixed-type.
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  • Composite lymphoma cosisting of mantle cell lymphoma and follicular lymphoma
    Akihito MATSUOKA, Tomoyuki TSUSHIMA, Masaki TANIBUCHI, Naomi KATSUKI, ...
    2013 Volume 54 Issue 11 Pages 2056-2061
    Published: 2013
    Released on J-STAGE: December 04, 2013
    DOIhttps://doi.org/10.11406/rinketsu.54.2056
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    Herein, we report the case of a 56-year-old man with composite lymphoma (CL) comprised of mantle cell lymphoma (MCL) and follicular lymphoma (FL). Six months after developing a right brachial tumor, he was diagnosed as having grade 3 FL with normal-size mantle zone. Simultaneously, advanced stage MCL with a diffuse growth pattern in a sigmoid colon tumor and abnormal lymphoid cells in bone marrow were observed. Thereafter, the right brachial tumor was re-examined and its mantle zone cells were immunophenotypically positive for cyclin D1 (CCND1) and cytogenetically positive for the IgH-CCND1 fusion gene. Consequently, he was diagnosed with composite lymphoma (CL) comprised of FL and MCL. As MCL and FL may form CL, the possible complication of MCL should be considered and steps taken to detect MCL.
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  • Composite lymphoma consisting of adult T-cell leukemia-lymphoma and diffuse large B-cell lymphoma
    Yuki NISHIDA, Yohei HYAKUNA, Kiyoshi FUJISAWA, Yujiro YAMANO, Koichi O ...
    2013 Volume 54 Issue 11 Pages 2062-2067
    Published: 2013
    Released on J-STAGE: December 04, 2013
    DOIhttps://doi.org/10.11406/rinketsu.54.2062
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    A 68-year-old man was admitted to our hospital because of left back pain and systemic lymphadenopathy with hypercalcemia. Serum anti-HTLV-1 antibody was positive. Left cervical lymph node (LN) biopsy revealed proliferation of medium-sized to large CD4-positive atypical cells with modest infiltration of CD20 and Epstein-Barr virus (EBV)-encoded RNA dual-positive atypical large cells. Monoclonal integration of HTLV-1 proviral DNA, plus clonal rearrangement of the T-cell receptor chain gene and the immunoglobulin heavy chain gene, were detected in the same LN specimen. Composite lymphoma consisting of adult T-cell leukemia/lymphoma (ATL) and EBV positive diffuse large B-cell lymphoma (DLBCL) was diagnosed. He was successfully treated with aggressive chemotherapy including rituximab and attained remission. However, eight months later, he developed right shoulder pain due to multiple bone invasions with bilateral cervical lymphadenopathy. Biopsies of a bone lesion and cervical LN revealed recurrence of ATL alone. The patient died despite salvage chemoradiotherapy. These findings suggest that ATL-related immunodeficiency might induce EBV-associated DLBCL.
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  • Follicular lymphoma complicated with myelofibrosis and macroglobulinemia at initial presentation
    Hiroyuki KURODA, Tomoyuki ABE, Wataru JOMEN, Masahiro YOSHIDA, Teppei ...
    2013 Volume 54 Issue 11 Pages 2068-2073
    Published: 2013
    Released on J-STAGE: December 04, 2013
    DOIhttps://doi.org/10.11406/rinketsu.54.2068
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    A 49-year-old woman presented with pharyngeal and cervical lymph node swelling in December 2010. Biopsy of the pharynx demonstrated follicular lymphoma which secreted large volumes of immunoglobulin M (IgM) and transforming growth factor-β (TGF-β). Bone marrow aspiration yielded a dry tap, and bone marrow biopsy demonstrated myelofibrosis associated with lymphoma cells on admission. The plasma concentration of TGF-β was elevated and monoclonal IgM gammopathy was detected. After only one course of chemotherapy with CHOP plus rituximab, remission of both lymphoma and myelofibrosis was achieved. Bone marrow aspiration became possible, and TGF-β and IgM levels normalized. Thus, the myelofibrosis was reversible.
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  • Detection of BCR-ABL1 chimeric gene-positive neutrophils in a patient with mixed phenotype acute leukemia
    Fusako NAGASAWA, Yukitsugu NAKAMURA, Katsuya TOKITA, Wataru TAKAHASHI, ...
    2013 Volume 54 Issue 11 Pages 2074-2078
    Published: 2013
    Released on J-STAGE: December 04, 2013
    DOIhttps://doi.org/10.11406/rinketsu.54.2074
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    We experienced two patients with mixed phenotype acute leukemia with t(9;22)(q34;q11.2); BCR-ABL1 according to the WHO classification 2008. The type of BCR/ABL1 was major in both patients, and the chimeric gene was also detected in neutrophils from peripheral blood by the fluorescence in situ hybridization technique. Patient 1 was a 59-year-old Japanese woman, and patient 2 a 45-year-old Japanese man. They had both developed leukemia suddenly. Their leukemic blasts expressed B cell and myeloid cell antigens, but concomitantly in patient 1 (biphenotypic) and separately in patient 2 (biclonal). Percentages of BCR-ABL1-positive neutrophils were 98% and 89%, respectively. Both patients received an imatinib (600 mg/day)-combined Hyper-CVAD regimen as induction therapy, followed by treatment with dasatinib (140 mg/day). MEC therapy was also applied between these two treatments in patient 2. At present, patient 1 has obtained complete molecular remission quantitatively and qualitatively, and patient 2 only quantitatively. Considering their acute onsets with no prior history of chronic myelocytic leukemia (CML), they were both diagnosed as having acute leukemia with Ph1, but not blastic crisis of CML. In this tyrosine kinase inhibitor era, it has become more difficult to differentiate these two types of Ph1-positive leukemia development.
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