Clinical features and treatment of multicentric Castleman׳s disease: a retrospective study of 21 Japanese patients at a single institute

DOI PubMed 被引用文献15件 参考文献62件
  • Kawabata Hiroshi
    Department of Hematology and Oncology Graduate School of Medicine, Kyoto University
  • Kadowaki Norimitsu
    Department of Hematology and Oncology Graduate School of Medicine, Kyoto University
  • Nishikori Momoko
    Department of Hematology and Oncology Graduate School of Medicine, Kyoto University
  • Kitawaki Toshio
    Department of Hematology and Oncology Graduate School of Medicine, Kyoto University
  • Kondo Tadakazu
    Department of Hematology and Oncology Graduate School of Medicine, Kyoto University
  • Ishikawa Takayuki
    Department of Hematology and Oncology Graduate School of Medicine, Kyoto University
  • Yoshifuji Hajime
    Department of Rheumatology and Clinical Immunology Graduate School of Medicine, Kyoto University
  • Yamakawa Noriyuki
    Department of Rheumatology and Clinical Immunology Graduate School of Medicine, Kyoto University
  • Imura Yoshitaka
    Department of Rheumatology and Clinical Immunology Graduate School of Medicine, Kyoto University
  • Mimori Tsuneyo
    Department of Rheumatology and Clinical Immunology Graduate School of Medicine, Kyoto University
  • Matsumura Yumi
    Department of Dermatology Graduate School of Medicine, Kyoto University
  • Miyachi Yoshiki
    Department of Dermatology Graduate School of Medicine, Kyoto University
  • Matsubara Takeshi
    Department of Nephrology Graduate School of Medicine, Kyoto University
  • Yanagita Motoko
    Department of Nephrology Graduate School of Medicine, Kyoto University
  • Haga Hironori
    Department of Diagnostic Pathology Graduate School of Medicine, Kyoto University
  • Takaori-Kondo Akifumi
    Department of Hematology and Oncology Graduate School of Medicine, Kyoto University

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タイトル別名
  • Clinical Features and Treatment of Multicentric Castleman's Disease : A Retrospective Study of 21 Japanese Patients at a Single Institute

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抄録

Multicentric Castleman's disease (MCD) is a rare polyclonal lymphoproliferative disorder that manifests with lymphadenopathy and inflammatory symptoms. In order to clarify the clinical features and actual management of MCD in Japan, we analyzed 21 patients diagnosed with MCD and treated in Kyoto University Hospital between 2005 and 2012. There were 12 men and 9 women. The median age at disease onset was 46 years, and the median follow-up period was 98 months. Common symptoms included splenomegaly (13/20), renal dysfunction (11/21), interstitial pneumonia (7/21), pleural effusion and/or ascites (7/21), and thrombocytopenia (6/21). The results of the anti-human immunodeficiency virus antibody and human herpes virus-8 DNA tests in the blood were available in 13 and 5 cases, respectively, and no patient was positive for either. Among 12 patients treated with tocilizumab, an anti-interleukin-6 receptor antibody, 11 exhibited an improvement in MCD-related symptoms and 3 achieved complete resolution of all these symptoms. In 8 patients treated with tocilizumab for over 1 year, the mean Hb level increased from 7.4 to 12.2 g/dL while the mean serum C-reactive protein level decreased from 13.2 to 0.4 mg/dL. Three patients died during the observation period due to sepsis, secondary leukemia, or pancreatic cancer. The clinical courses of most cases were indolent; however, in some cases with pleural effusion, ascites, renal dysfunction, and/or thrombocytopenia, the disease manifestation was serious. A nationwide survey is required to further clarify the epidemiology, clinical features, and optimal treatment strategies of MCD in Japan. [J Clin Exp Hematop 53(1): 69-77, 2013]

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