著者

Yoshito Nishimura Asami Nishikori Haruki Sawada Torrey Czech Yuki Otsuka Midori Filiz Nishimura Hiroki Mizuno Naoki Sawa Shuji Momose Kumiko Ohsawa Fumio Otsuka Yasuharu Sato

出版者

The Japanese Society for Lymphoreticular Tissue Research

雑誌

Journal of Clinical and Experimental Hematopathology (ISSN:13464280)

巻号頁・発行日

pp.21038, (Released:2022-03-05)

参考文献数

38

被引用文献数

4

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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.