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Otsuka, Mizuna Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences
Koga, Tomohiro Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences
Sumiyoshi, Remi Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences
Fukui, Shoichi Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences
Kaneko, Yuko Division of Rheumatology, Department of Internal Medicine Keio University School of Medicine Tokyo Japan
Shimizu, Takayuki Division of Hematology, Department of Medicine, Keio University School of Medicine
Katsube, Atsushi Division of Clinical Oncology and Hematology, Department of Internal Medicine, The Jikei University School of Medicine
Yano, Shingo Division of Clinical Oncology and Hematology, Department of Internal Medicine, The Jikei University School of Medicine
Masaki, Yasufumi Department of Hematology and Immunology, Kanazawa Medical University
Ide, Makoto Department of Hematology, Takamatsu Red Cross Hospital
Yoshifuji, Hajime DDepartment of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University
Kitano, Masayasu Department of Collagen Disease and Rheumatology, Sumitomo Hospital
Sato, Yasuharu Department of Molecular Hematopathology, Okayama University Graduate School of Health Sciences ORCID Kaken ID researchmap
Sawa, Naoki Nephrology Center and Department of Rheumatology, Toranomon Hospital Kajigaya
Niiro, Hiroaki Department of Clinical Immunology and Rheumatology/Infectious Disease, Kyushu University Hospital
Nakamura, Naoya Department of Pathology, Tokai University School of Medicine
Fajgenbaum, David C. Center for Cytokine Storm Treatment & Laboratory, University of Pennsylvania
van Rhee, Frits Myeloma Center, University of Arkansas for Medical Sciences
Kawakami, Atsushi Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences
Abstract
Individuals diagnosed with Castleman disease (CD) and TAFRO syndrome (characterized by thrombocytopenia, anasarca, fever, bone marrow fibrosis, and organomegaly) displays a wide range of clinical symptoms, including varying patterns of lymph node enlargement, systemic inflammation, and impaired organ function. Some patients may present with both CD and TAFRO syndrome concurrently. A retrospective study conducted across multiple centers in Japan examined 321 cases to determine if the quantity and position of swollen lymph nodes could forecast the clinical progression and intensity of these conditions. Interestingly, the study revealed that patients with TAFRO syndrome exhibited lymphadenopathy across all ranges of lymph node region counts. Moreover, no specific clinical patterns were associated with the number of affected lymph node regions in CD patients, regardless of whether they also had TAFRO syndrome. These results enhance our understanding of the clinical variability in CD and TAFRO syndrome, suggesting that a comprehensive clinical evaluation, rather than relying solely on lymph node count, is crucial for effectively managing these conditions. Additional studies are required to establish reliable diagnostic markers and to predict disease severity at the time of diagnosis, ultimately improving patient outcomes.
Keywords
Castleman disease
clinical variability
lymph node distribution
multicenter cohort
TAFRO syndrome
Published Date
2025-01-25
Publication Title
American Journal of Hematology
Volume
volume100
Issue
issue4
Publisher
Wiley
Start Page
592
End Page
605
ISSN
0361-8609
NCID
AA00520673
Content Type
Journal Article
language
English
OAI-PMH Set
岡山大学
Copyright Holders
© 2025 The Author(s).
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DOI
Web of Science KeyUT
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isVersionOf https://doi.org/10.1002/ajh.27612
License
http://creativecommons.org/licenses/by/4.0/
Citation
Otsuka, M., Koga, T., Sumiyoshi, R., Fukui, S., Kaneko, Y., Shimizu, T., Katsube, A., Yano, S., Masaki, Y., Ide, M., Yoshifuji, H., Kitano, M., Sato, Y., Sawa, N., Niiro, H., Nakamura, N., Fajgenbaum, D.C., van Rhee, F. and Kawakami, A. (2025), Exploring the Clinical Diversity of Castleman Disease and TAFRO Syndrome: A Japanese Multicenter Study on Lymph Node Distribution Patterns. Am J Hematol, 100: 592-605. https://doi.org/10.1002/ajh.27612
助成情報
23K15352: キャッスルマン病の国際的なコンセンサス構築に向けた課題を解決する基盤研究 ( 独立行政法人日本学術振興会 / Japan Society for the Promotion of Science )
( 厚生労働省 / Ministry of Health )