Yi Ping, Zhang Xing-Xing, Tang Tian, Wang Ying, Wu Xiao-Chuan, Li Xing-Fang
Department of Pediatrics, The Second Xiangya Hospital of Central South University, Changsha 410011, China.
Zhongguo Dang Dai Er Ke Za Zhi. 2025 May 15;27(5):613-617. doi: 10.7499/j.issn.1008-8830.2412050.
The patient, an 11-year-old girl, was admitted with recurrent fever for 20 days, worsening with abdominal distension for 7 days. Upon admission, she presented with recurrent fever, lymphadenopathy, hepatosplenomegaly, polyserositis, and multiple organ dysfunction. Lymph node pathology and clinical manifestations confirmed the diagnosis of idiopathic multicentric Castleman disease-TAFRO syndrome. Treatment with siltuximab combined with glucocorticoids was initiated, followed by maintenance therapy with tocilizumab. The patient is currently in complete clinical remission. Therefore, once a child is diagnosed with idiopathic multicentric Castleman disease -TAFRO syndrome, early use of siltuximab should be considered for rapid disease control, followed by tocilizumab for maintenance therapy.
该患者为一名11岁女孩,因反复发热20天入院,伴腹胀加重7天。入院时,她表现为反复发热、淋巴结病、肝脾肿大、多浆膜炎和多器官功能障碍。淋巴结病理及临床表现确诊为特发性多中心Castleman病-TAFRO综合征。开始使用西妥昔单抗联合糖皮质激素治疗,随后用托珠单抗进行维持治疗。患者目前处于完全临床缓解状态。因此,一旦儿童被诊断为特发性多中心Castleman病-TAFRO综合征,应考虑早期使用西妥昔单抗以快速控制病情,随后用托珠单抗进行维持治疗。