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Tentative diagnostic criteria and disease severity classification for Castleman disease: A report of the research group on Castleman disease in Japan |
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| Authors: | Shino Fujimoto Tomohiro Koga Hiroshi Kawabata Shinichiro Okamoto Masao Mizuki |
| Institution: | 1. Division of Hematology and Immunology, Medicine, Kanazawa Medical University, Ishikawa, Japan;2. Unit of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan;3. Center for Bioinformatics and Molecular Medicine, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan;4. Division of Hematology, Department of Medicine, Keio University School of Medicine, Tokyo, Japan;5. Chemotherapy and Oncology Center, Osaka University Hospital, Osaka, Japan |
| Abstract: | Objectives: To determine the tentative diagnostic criteria and disease severity classification for Castleman disease (CD) and describe the clinical and pathologic features among human herpesvirus 8 (HHV-8) negative idiopathic multicentric CD (iMCD) in the Japanese population. Methods: We established the working groups for the research of CD in Japan and had meetings to discuss and define the tentative diagnostic criteria and disease severity classification for CD. We subsequently analyzed 142 patients classified into iMCD by using the nationwide Japanese patient registry. Results: We proposed the preliminary diagnostic criteria and disease severity classification for CD based on our discussion. In addition, we made a proposal for the disease activity score. We identified clinical and pathological features of patients with iMCD diagnosed by these diagnostic criteria. In the disease severity classification, 37, 33 and 30% patients were categorized into mild, moderate and severe diseases, respectively. Conclusion: This is the first proposal for diagnosis and classification of CD by the Japanese group. Further studies are required to validate whether they can distinguish CD from other inflammatory diseases and to determine their sensitivity and specificity. |
| Keywords: | Castleman disease idiopathic multicentric Castleman disease diagnostic criteria disease severity classification TAFRO syndrome |