Castleman-Kojima Disease (TAFRO Syndrome) : A Novel Systemic Inflammatory Disease Characterized by a Constellation of Symptoms, Namely, Thrombocytopenia, Ascites (Anasarca), Microcytic Anemia, Myelofibrosis, Renal Dysfunction, and Organomegaly : A Statu
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概要
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Recently, a unique clinicopathologic variant of multicentric Castleman's disease (MCD) has been identified in Japan. This disease is characterized by a constellation of symptoms, as listed in the title, and multiple lymphadenopathy of mild degree with a pathologic diagnosis of atypical CD, often posing diagnostic and therapeutic problems for pathologists and hematologists, respectively. These findings suggest that this disease represents a novel clinical entity belonging to systemic inflammatory disorders with a background of immunological abnormality beyond the ordinal spectrum of MCD. To define this disorder more clearly, Japanese participants presented clinicopathologic data at the Fukushima and Nagoya meetings. Many of the patients presented by the participants were significantly accompanied by a combination of thrombocytopenia, ascites (anasarca), pleural effusions, microcytic anemia, fever, myelofibrosis, renal dysfunction, and organomegaly (TAFRO). Multiple lymphadenopathies were generally of mild degree, less than 1.5 cm in diameter, and consistently featured the histopathology of mixed- or less hyaline vascular-type CD. Autoantibodies were often detected. However, this disease did not fulfill the diagnostic criteria for well-known autoimmune diseases including systemic lupus erythematosus. Castleman-Kojima disease and TAFRO syndrome (the favored clinical term) were proposed for this disease. The patients were sensitive to steroid and anti-interleukin-6 receptor antibody (tocilizumab), but some exhibited a deteriorated clinical course despite the treatment. The participants proposed a future nationwide survey and a Japanese consortium to facilitate further clinical and therapeutic studies of this novel disease. [<I>J Clin Exp Hematop 53(</I><I>1): 57-61,</I> <I>2013</I>]
著者
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MASAKI Yasufumi
Hematology & Immunology, Kanazawa Medical University School of Medicine
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Kojima Masaru
Department Of Anatomic And Diagnostic Pathology Dokkyo Medical University
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Nakamura Naoya
Department Of Hematology Niigata University Graduate School Of Medical And Dental Sciences
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KINOSHITA Tomohiro
Department of Hematology and Cell Therapy, Aichi Cancer Center
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Kawabata Hiroshi
Department of Electronic Science and Engineering, Kyoto University, Kyoto 606-8501, Japan
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NAKAMURA Shigeo
Department of Applied Chemistry, Faculty of Engineering, Kanagawa University
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Takai Kazue
Division of Hematology, Niigata City General Hospital
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Masaki Yasufumi
Hematology and Immunology, Kanazawa Medical University
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Aoki Sadao
Clinical Oncology Division, Faculty of Pharmaceutical Sciences, Niigata University of Pharmacy and Applied Life Sciences
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Kinoshita Tomohiro
Department of Hematology and Cell Therapy, Aichi Cancer Center Hospital
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