ID | 63521 |
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Nishimura, Yoshito
Department of General Medicine, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences
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Nishikori, Asami
Division of Pathophysiology, Okayama University Graduate School of Health Sciences
Sawada, Haruki
Department of Medicine, University of Hawaii
Czech, Torrey
Department of Medicine, University of Hawaii
Otsuka, Yuki
Department of General Medicine, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences
Nishimura, Midori Filiz
Department of Pathology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences
Mizuno, Hiroki
Nephrology Center, Toranomon Hospital Kajigaya
Sawa, Naoki
Nephrology Center, Toranomon Hospital Kajigaya
Momose, Shuji
Department of Pathology, Saitama Medical Center, Saitama Medical University
Ohsawa, Kumiko
Division of Pathophysiology, Okayama University Graduate School of Health Sciences
Otsuka, Fumio
Department of General Medicine, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences
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Sato, Yasuharu
Division of Pathophysiology, Okayama University Graduate School of Health Sciences
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Abstract | Idiopathic multicentric Castleman disease (iMCD) is a systemic disorder characterized by systemic inflammation and organ dysfunction associated with an increase in pro-inflammatory cytokines. Some patients with iMCD are positive for autoantibodies, although their significance and relationship with specific associated autoimmune diseases are unclear. This study retrospectively analyzed the clinicopathological features of iMCD patients focusing on autoantibodies. Among 63 iMCD patients in our database, 19 were positive for at least one autoantibody. Among the 19, we identified five with plasma cell type (PC)-iMCD lymph node histopathology and positive anti-phospholipid antibodies. These patients were likely to have thrombocytopenia, anasarca, fever, reticulin fibrosis or renal insufficiency, organomegaly (TAFRO) symptoms, and thrombotic events. The present study suggests that patients with undiagnosed or atypical autoimmune diseases, including anti-phospholipid syndrome (APS), were treated for iMCD. APS may present with thrombocytopenia or even multi-organ failure, which overlap with clinical presentations of iMCD. Due to differences in the treatment regimen and follow-up, recognition of the undiagnosed autoimmune disease process in those suspected of iMCD is essential. Our study highlights the importance of complete exclusion of differential diagnoses in patients with iMCD in their diagnostic workup.
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Keywords | idiopathic plasmacytic lymphadenopathy
multicentric Castleman disease
autoimmune
anti-phospholipid syndrome
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Published Date | 2022
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Publication Title |
Journal Of Clinical And Experimental Hematopatholo
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Publisher | The Japanese Society for Lymphoreticular Tissue Research
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Start Page | 21038
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ISSN | 1346-4280
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Content Type |
Journal Article
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language |
English
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OAI-PMH Set |
岡山大学
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Copyright Holders | © 2022 The Japanese Society for Lymphoreticular Tissue Research
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File Version | publisher
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Related Url | isVersionOf https://doi.org/10.3960/jslrt.21038
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License | https://creativecommons.org/licenses/by-nc-sa/4.0/
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