Qiu Yuchong, Zhang Miaoyan, Jia Congwei, Zhang Lu, Li Jian
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
4+4 Medical Doctor Program, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
Ann Hematol. 2025 Apr;104(4):2559-2561. doi: 10.1007/s00277-025-06324-y. Epub 2025 Mar 26.
Idiopathic multicentric Castleman disease (iMCD)- TAFRO (thrombocytopenia, ascites, fever, reticulin fibrosis and organomegaly) is a clinically severe situation characterized by cytokine storms that are potentially fatal, necessitating prompt and decisive medical intervention. The International Castleman's Disease Collaborative Network (CDCN) prioritizes the combination of long-term siltuximab and high-dose steroids as the preferred therapeutic option for iMCD-TAFRO. Here, we report a rare case of spontaneous remission in iMCD-TAFRO. In this case, the patient diagnosed with iMCD-TAFRO experienced alleviation of clinical symptoms and normalization of laboratory test results without undergoing any etiological treatment. This case sheds new light on the therapeutic strategies for iMCD-TAFRO. Following the stabilization of the onset cytokine storm, a detailed evaluation of the patient's condition should be performed to determine the need for continuous medical treatment.
特发性多中心Castleman病（iMCD）-TAFRO（血小板减少、腹水、发热、网状纤维增生和器官肿大）是一种临床严重情况，其特征为细胞因子风暴，有潜在致命性，需要迅速而果断的医学干预。国际Castleman病协作网络（CDCN）将长期使用司妥昔单抗和高剂量类固醇联合作为iMCD-TAFRO的首选治疗方案。在此，我们报告一例iMCD-TAFRO自发缓解的罕见病例。在该病例中，被诊断为iMCD-TAFRO的患者临床症状缓解，实验室检查结果恢复正常，且未接受任何病因治疗。该病例为iMCD-TAFRO的治疗策略提供了新的思路。在起始细胞因子风暴稳定后，应详细评估患者病情以确定是否需要持续治疗。
