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异基因造血细胞移植治疗Richter综合征:单中心经验

Allogeneic Hematopoietic Cell Transplantation for Richter Syndrome: A Single-Center Experience.

作者信息

Kharfan-Dabaja Mohamed A, Kumar Ambuj, Stingo Facundo E, Khimani Farhad, Hussaini Mohammad, Ayala Ernesto, Nishihori Taiga, Shah Bijal, Locke Frederick L, Pinilla-Ibarz Javier, Chavez Julio C

机构信息

Department of Blood and Marrow Transplantation and Cellular Immunotherapy, Moffitt Cancer Center, Tampa, FL.

Program for Comparative Effectiveness Research, University of South Florida, Morsani College of Medicine, Tampa, FL.

出版信息

Clin Lymphoma Myeloma Leuk. 2018 Jan;18(1):e35-e39. doi: 10.1016/j.clml.2017.10.002. Epub 2017 Oct 12.

Abstract

BACKGROUND

Recent studies have shown dismal outcomes when chronic lymphocytic leukemia progresses to Richter syndrome after patients receive ibrutinib, with a median overall survival ranging from 2.6 to 3.5 months. Published data on efficacy of allogeneic hematopoietic cell transplantation in Richter syndrome are limited to single-center case series and registry data.

PATIENTS AND METHODS

We evaluated the efficacy of allogeneic transplantation in 10 patients, median age of 63 (range, 50-74) years, allografted at a median of 5 (range, 4-25) months from diagnosis of Richter syndrome. All showed an objective response to therapy before transplantation (first complete remission = 7 [70%], first partial response = 2 [20%], second partial response = 1 [10%]). Most received a myeloablative conditioning regimen (n = 7, 70%). Filgrastim-mobilized peripheral blood stem cells was the preferred cell source (n = 10, 100%).

RESULTS

Median follow-up of surviving patients was 46 (range, 15-82) months. The 4-year overall survival was 50% (95% confidence interval [CI], 19%-81%). Nonrelapse mortality at 1 year and 4 years post-transplantation were 40% (95% CI, 19%-85%) for both time points. The 4-year incidence of relapse/progression was 10% (95% CI, 2%-64%).

CONCLUSION

Allogeneic hematopoietic cell transplantation is an effective treatment for patients with Richter syndrome who show an objective response before allografting. Patients must be referred to transplant centers as soon as the diagnosis is confirmed to evaluate candidacy for the procedure and identify a suitable donor in a timely manner.

摘要

背景

近期研究表明,慢性淋巴细胞白血病患者接受依鲁替尼治疗后进展为 Richter 综合征时预后不佳,中位总生存期为 2.6 至 3.5 个月。关于异基因造血细胞移植治疗 Richter 综合征疗效的已发表数据仅限于单中心病例系列和登记数据。

患者与方法

我们评估了 10 例患者接受异基因移植的疗效,这些患者的中位年龄为 63 岁(范围 50 - 74 岁),自 Richter 综合征诊断起至接受移植的中位时间为 5 个月(范围 4 - 25 个月)。所有患者在移植前对治疗均有客观反应(首次完全缓解 = 7 例 [70%],首次部分缓解 = 2 例 [20%],第二次部分缓解 = 1 例 [10%])。大多数患者接受了清髓性预处理方案(n = 7,70%)。非格司亭动员的外周血干细胞是首选的细胞来源(n = 10,100%)。

结果

存活患者的中位随访时间为 46 个月(范围 15 - 82 个月)。4 年总生存率为 50%(95%置信区间 [CI],19% - 81%)。移植后 1 年和 4 年的非复发死亡率在两个时间点均为 40%(95% CI,19% - 85%)。4 年复发/进展发生率为 10%(95% CI,2% - 64%)。

结论

异基因造血细胞移植是对移植前有客观反应的 Richter 综合征患者的有效治疗方法。一旦确诊,患者必须尽快转诊至移植中心,以评估该治疗的适用性并及时确定合适的供体。

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