Department of Hematology, the First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, 310003, China.
Front Med. 2017 Jun;11(2):287-292. doi: 10.1007/s11684-017-0510-2. Epub 2017 Apr 3.
Castleman disease (CD) is uncommon non-clonal lymphoproliferative disorder with unknown etiology. No standard therapy is recommended for relapsed/refractory CD patients, thus requiring development of novel experimental approaches. Our cohort of three adult patients with multicentric CD (MCD) were treated with refractory to traditional chemotherapy lenalidomide-containing regimens (10-25 mg lenalidomide perorally administered on days 1-21 in 28-day cycle) as second- to fourth-line treatment. Partial remission was achieved in first plasma-cell CD patient, who relapsed seven months after autologous hematopoietic stem cell transplantation and then failed to respond to four cycles of chemotherapy. Partial remission was obtained in second patient with CD and polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome. Third case showed complete remission with complete disappearance of pleural effusion and ascites and normalization of platelet count. To conclude, encouraging clinical responses were achieved in cohort of three patients with lenalidomide-based regimen, though long-term efficacy remains to be observed.We propose further investigation of therapeutic potential of this drug in treating MCD.
卡斯尔曼病（CD）是一种罕见的非克隆性淋巴组织增生性疾病，病因不明。对于复发性/难治性 CD 患者，目前尚无推荐的标准治疗方法，因此需要开发新的实验方法。我们的三组成年多中心 CD（MCD）患者接受了难治性传统化疗联合来那度胺方案（28 天周期内，第 1-21 天每天口服 10-25mg 来那度胺）治疗，作为二线至四线治疗。第一个浆细胞 CD 患者在接受自体造血干细胞移植后 7 个月复发，随后对 4 个周期的化疗无反应，该患者获得部分缓解。第二位患有 CD 和多发性神经病、器官肿大、内分泌病、单克隆丙种球蛋白病和皮肤改变综合征的患者获得部分缓解。第三位患者的完全缓解表现为胸腔积液和腹水完全消失，血小板计数恢复正常。总之，在接受来那度胺为基础的方案治疗的三组患者中取得了令人鼓舞的临床反应，但长期疗效仍有待观察。我们建议进一步研究该药物在治疗 MCD 中的治疗潜力。
