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- Takashi Ozaki, Chiharu Imada, Kohei Shirakami, Tasuku Abe, Yuta Shingai, Tatsuhito Umeki, Akihiro Fukuda, Koji Ishii, and Hirotaka Shibata.
- Department of Endocrinology, Metabolism, Rheumatology and Nephrology, Faculty of Medicine, Oita University, Japan.
- Intern. Med. 2025 Feb 8.
AbstractIdiopathic multicentric Castleman disease (iMCD) accompanied by thrombocytopenia, anasarca, fever, reticulin myelofibrosis/renal insufficiency, and organomegaly (TAFRO) frequently develops into prolonged severe thrombocytopenia and multiorgan dysfunction, which is associated with a high mortality rate. We herein describe three cases of iMCD-TAFRO in which serum soluble interleukin-2 receptor (sIL-2R) levels varied in relation to disease activity following the initiation of treatment. Thrombocytopenia persisted for several weeks to months after the normalization of the patients' C-reactive protein levels, whereas their sIL-2R levels normalized 10-47 days prior to platelet recovery (≥100×109/L). The serum sIL-2R measurement may therefore be an effective method for evaluating the disease activity in iMCD-TAFRO patients with prolonged thrombocytopenia.
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