Castleman disease (CD) is a rare lymphoproliferative disease characterized by diverse clinical and pathologic features. Due to its rarity, there are limited studies comparing currently available therapies. The role of autologous stem cell transplantation (ASCT) in CD has not yet been established. In this paper, we describe the clinical characteristics, treatment choices, and outcomes in 34 Mayo Clinic patients diagnosed with multicentric CD from July 1, 2003 to April 30, 2018. Eighteen patients (53%) also met the criteria for POEMS, including 14 with the osteosclerotic variant. The first-line treatments included: steroid monotherapy (4), cytotoxic chemotherapy (6), rituximab alone (8) or with chemotherapy (2), anti-IL6 treatment (3), and ASCT (10). The median follow-up was 4.8 (range: 0.1–15.2) years. The 5- and 10-year overall survival rates were 84% and 71%, respectively. Sixteen patients received high-dose chemotherapy followed by ASCT during their disease course. Among those, 14 had multicentric CD associated with POEMS. There were no transplant-related deaths. All patients had at least a partial response to ASCT, most of whom achieved a complete response. The favorable outcomes seen with ASCT in this cohort suggest that transplantation may have a role in multicentric CD, particularly for patients with multicentric CD associated with POEMS.

中文翻译：

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多中心Castleman病：以自体干细胞移植为重点的单中心治疗经验

Castleman 病 (CD) 是一种罕见的淋巴组织增生性疾病，具有多种临床和病理特征。由于其稀有性，比较当前可用疗法的研究有限。自体干细胞移植 (ASCT) 在 CD 中的作用尚未确定。在本文中，我们描述了 2003 年 7 月 1 日至 2018 年 4 月 30 日期间被诊断为多中心 CD 的 34 名 Mayo Clinic 患者的临床特征、治疗选择和结果。18 名患者 (53%) 也符合 POEMS 标准，其中 14 名与骨硬化变体。一线治疗包括：类固醇单药治疗 (4)、细胞毒性化疗 (6)、利妥昔单抗单独治疗 (8) 或联合化疗 (2)、抗 IL6 治疗 (3) 和 ASCT (10)。中位随访时间为 4.8（范围：0.1-15.2）年。5 年和 10 年总生存率分别为 84% 和 71%。16 名患者在病程中接受了大剂量化疗，随后进行了 ASCT。其中，14 人患有与 POEMS 相关的多中心 CD。没有与移植相关的死亡。所有患者对 ASCT 至少有部分反应，其中大多数达到完全反应。在该队列中使用 ASCT 观察到的有利结果表明移植可能在多中心 CD 中起作用，特别是对于与 POEMS 相关的多中心 CD 患者。他们中的大多数人都取得了完全的回应。在该队列中使用 ASCT 观察到的有利结果表明移植可能在多中心 CD 中起作用，特别是对于与 POEMS 相关的多中心 CD 患者。他们中的大多数人都取得了完全的回应。在该队列中使用 ASCT 观察到的有利结果表明移植可能在多中心 CD 中起作用，特别是对于与 POEMS 相关的多中心 CD 患者。