{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T16:18:13Z","timestamp":1792167493699,"version":"4.4.0"},"reference-count":26,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2026,1,10]],"date-time":"2026-01-10T00:00:00Z","timestamp":1768003200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,1,10]],"date-time":"2026-01-10T00:00:00Z","timestamp":1768003200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["82470150"],"award-info":[{"award-number":["82470150"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2026,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Idiopathic multicentric Castleman disease (iMCD)\u2010TAFRO (Thrombocytopenia, Anasarca, Fever, Reticulin fibrosis\/renal dysfunction, Organomegaly) is the most severe clinical subtype of iMCD characterized by a catastrophic cytokine storm. Currently, iMCD\u2010TAFRO is regarded as incurable which needs \u201cindefinite\u201d treatment. However, long\u2010term data are sparse, and treatment discontinuation may be possible in a subset of patients. This multicenter, retrospective study analyzed 27 patients who discontinued treatment. All patients had biochemical complete response (CR) at the time of drug discontinuation. Most patients were treated with myeloma\u2010like treatment (59.3%) and only 11.1% received anti\u2010IL\u20106 therapy. With a median follow\u2010up of 31\u2009months (range, 12\u2013108) after treatment discontinuation, only four patients (14.8%) suffered from progression of disease (PD) and the others (85.2%) maintained biochemical CR. Patients who suffered from PD had a significantly lower pretreatment CRP level than patients maintaining responses (43.7\u2009\u00b1\u200939.3 vs. 126.4\u2009\u00b1\u200962.5\u2009mg\/L,\n                    <jats:italic>p<\/jats:italic>\n                    \u2009=\u20090.018). No significant impacts of different treatment approaches or duration of treatment on the likelihood of achieving long\u2010term remission were observed. The median time between drug discontinuation to PD (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u20094) was 9.5\u2009months (range, 5\u201312). These patients all achieved treatment responses again with the same or similar treatment approaches. The estimated PFS at 6\u2009months, 1\u2009year, and 3\u2009years were 96.3%, 85.2%, and 85.2%, respectively. All patients remained alive with a median of 31\u2009months follow\u2010up after drug discontinuation. This study suggests that some iMCD\u2010TAFRO patients might maintain long\u2010term remission after treatment discontinuation. For patients who achieved biochemical CR, an attempt at treatment discontinuation could be considered.\n                  <\/jats:p>","DOI":"10.1002\/ajh.70199","type":"journal-article","created":{"date-parts":[[2026,1,10]],"date-time":"2026-01-10T10:54:58Z","timestamp":1768042498000},"page":"447-455","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Idiopathic Multicentric Castleman Disease\u2014\n                    <scp>TAFRO<\/scp>\n                    : A Potentially Curable Disease?"],"prefix":"10.1002","volume":"101","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0860-9625","authenticated-orcid":false,"given":"Lu","family":"Zhang","sequence":"first","affiliation":[{"name":"Department of Hematology, Peking Union Medical College Hospital Chinese Academy of Medical Sciences and Peking Union Medical College  Beijing China"},{"name":"Center for Rare Diseases, State Key Laboratory of Complex, Severe, and Rare Diseases, Peking Union Medical College Hospital Chinese Academy of Medical Sciences  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-7464-0446","authenticated-orcid":false,"given":"Jia\u2010ying","family":"Ge","sequence":"additional","affiliation":[{"name":"Department of Hematology, The First Affiliated Hospital, College of Medicine Zhejiang University  Hangzhou China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1654-3571","authenticated-orcid":false,"given":"Si\u2010yuan","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Hematology, Peking Union Medical College Hospital Chinese Academy of Medical Sciences and Peking Union Medical College  Beijing China"},{"name":"Center for Rare Diseases, State Key Laboratory of Complex, Severe, and Rare Diseases, Peking Union Medical College Hospital Chinese Academy of Medical Sciences  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5603-4160","authenticated-orcid":false,"given":"Hong\u2010yan","family":"Tong","sequence":"additional","affiliation":[{"name":"Department of Hematology, The First Affiliated Hospital, College of Medicine Zhejiang University  Hangzhou China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4575-3287","authenticated-orcid":false,"given":"Liang\u2010shun","family":"You","sequence":"additional","affiliation":[{"name":"Department of Hematology, The First Affiliated Hospital, College of Medicine Zhejiang University  Hangzhou China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4549-0694","authenticated-orcid":false,"given":"Jian","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Hematology, Peking Union Medical College Hospital Chinese Academy of Medical Sciences and Peking Union Medical College  Beijing China"},{"name":"Center for Rare Diseases, State Key Laboratory of Complex, Severe, and Rare Diseases, Peking Union Medical College Hospital Chinese Academy of Medical Sciences  Beijing China"},{"name":"State Key Laboratory of Common Mechanism Research for Major Diseases Chinese Academy of Medical Sciences and Peking Union Medical College  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,1,10]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2016-10-746933"},{"issue":"7","key":"e_1_2_10_3_1","first-page":"529","article-title":"The Consensus of the Diagnosis and Treatment of Castleman Disease in China (2021)","volume":"42","author":"Hematology Committee of Chinese Medical Association; Hematological Oncology Committee of China Anti\u2010Cancer Association; China Castleman Disease Network (CCDN)","year":"2021","journal-title":"Zhonghua Xue Ye Xue Za Zhi"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2018-07-862334"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.24242"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.26292"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.lanwpc.2023.100720"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra2026131"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V110.11.646.646"},{"issue":"4","key":"e_1_2_10_10_1","first-page":"283","article-title":"Rapid Relapse of Idiopathic Multicentric Castleman Disease After Siltuximab Discontinuation in a Case With Complete Remission for More Than 10\u2009Years","volume":"41","author":"Gao Y. 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