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异基因造血干细胞移植治疗蕈样肉芽肿和塞扎里综合征的结果。

Outcome of allogeneic hematopoietic stem cell transplantation for mycosis fungoides and Sézary syndrome.

机构信息

Division of Hematology, Department of Medicine, Keio University School of Medicine, Tokyo, Japan.

Department of Hematology, Hokkaido University Faculty of Medicine, Sapporo, Japan.

出版信息

Hematol Oncol. 2020 Aug;38(3):266-271. doi: 10.1002/hon.2719. Epub 2020 Mar 3.

Abstract

Although allogeneic hematopoietic stem cell transplantation (HSCT) has been reported to provide prolonged remission of relapsed/refractory mycosis fungoides (MF) and Sézary syndrome (SS), its role has not been fully evaluated. Here, the outcomes of allogeneic HSCT for patients with MF/SS were retrospectively evaluated by using the registry database of the Japan Society for Hematopoietic Cell Transplantation. Forty-eight patients were evaluable and enrolled in the analysis. Median age was 45.5 years. Eighteen patients (38%) received myeloablative conditioning, and 33 (69%) received HSCT from an alternative donor. Disease status was complete or partial response in 25% of the patients and relapsed or refractory in the others. At the time of analysis, 18 patients were alive, with a median follow-up of 31.0 months (range, 3.8-31.1). Three-year overall survival (OS) and progression-free survival (PFS) were 30% (95%CI, 16-45%) and 19% (95%CI, 9-31%), respectively. Disease progression was not observed later than 17 months after transplantation. Both disease status and performance status at transplant significantly affected OS and PFS. Although our findings suggest that allogeneic HSCT provides long-term PFS in patients with MF/SS, the timing of transplantation should be decided carefully based on the disease status and the patient's condition in order to improve the outcome.

摘要

虽然异体造血干细胞移植(HSCT)已被报道可延长复发/难治性蕈样真菌病(MF)和塞扎里综合征(SS)的缓解期,但它的作用尚未得到充分评估。在这里,我们使用日本造血细胞移植学会的注册数据库,回顾性地评估了异体 HSCT 治疗 MF/SS 患者的结果。48 例患者可评估并纳入分析。中位年龄为 45.5 岁。18 例(38%)接受了清髓性预处理,33 例(69%)接受了来自替代供者的 HSCT。25%的患者疾病状态为完全或部分缓解,其余患者为复发或难治性。在分析时,18 例患者存活,中位随访时间为 31.0 个月(范围为 3.8-31.1)。3 年总生存率(OS)和无进展生存率(PFS)分别为 30%(95%CI,16-45%)和 19%(95%CI,9-31%)。疾病进展在移植后 17 个月内未观察到。移植时的疾病状态和表现状态均显著影响 OS 和 PFS。尽管我们的研究结果表明,异体 HSCT 可为 MF/SS 患者提供长期的 PFS,但为了改善预后,应根据疾病状态和患者的情况谨慎决定移植时机。

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