Idiopathic 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 (≥100×10/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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