Zhou Xiao-Hui, Wang Xiao-Dong, Lin Qi-Hong, Wang Chun-Jing, Yang Chun-Lan, Li Yue, Zhang Xiao-Ling, Zhang Yu, Yu Yue, Liu Si-Xi
Department of Hematology and Oncology, Shenzhen Children's Hospital, Shenzhen, Guangdong 518000, China.
Zhongguo Dang Dai Er Ke Za Zhi. 2024 Dec 15;26(12):1301-1307. doi: 10.7499/j.issn.1008-8830.2406036.
To investigate the clinical characteristics of cytokine release syndrome (CRS) in children with thalassemia major (TM) after haploidentical hematopoietic stem cell transplantation (haplo-HSCT) and their prognosis.
A retrospective analysis was performed for the clinical data of 280 children with TM who underwent haplo-HSCT in the Department of Hematology and Oncology, Shenzhen Children's Hospital, from January 2019 to December 2021. According to the CRS criteria, they were divided into two groups: CRS grade <3 (260 children) and CRS grade ≥3 (20 children). The children with TM were analyzed in terms of clinical characteristics of CRS after haplo-HSCT and their prognosis.
There were significant differences between the two groups in neutrophil engraftment time, clinical manifestations of CRS, and the rate of use of glucocorticoids within 4 days after haplo-HSCT (=0.012, 0.040, and <0.001 respectively). For the CRS grade <3 group, the incidence rate of acute graft-versus-host disease (aGVHD) was 9.6% within 3 months after transplantation, while no aGVHD was observed in the CRS grade ≥3 group within 3 months after transplantation, but there was no significant difference in the incidence of aGVHD between the two groups within 3 months after transplantation (=0.146). No transplantation-related death was observed in either group within 3 months after haplo-HSCT.
The children with CRS grade≥3 have an early neutrophil engraftment time, severe and diverse clinical manifestations of CRS, and a high rate of use of glucocorticoids within 4 days after haplo-HSCT. For these children, early use of low-dose glucocorticoids after transplantation may alleviate CRS response and reduce the incidence of aGVHD, thereby bringing more benefits to the children. CRS after haplo-HSCT has no significant impact on the prognosis of the children.
探讨重型地中海贫血（TM）患儿单倍体造血干细胞移植（haplo-HSCT）后细胞因子释放综合征（CRS）的临床特征及其预后。
回顾性分析2019年1月至2021年12月在深圳市儿童医院血液肿瘤科接受haplo-HSCT的280例TM患儿的临床资料。根据CRS标准，将其分为两组：CRS<3级（260例患儿）和CRS≥3级（20例患儿）。对TM患儿haplo-HSCT后CRS的临床特征及其预后进行分析。
两组在中性粒细胞植入时间、CRS临床表现及haplo-HSCT后4天内糖皮质激素使用率方面存在显著差异（分别为P=0.012、0.040和<0.001）。对于CRS<3级组，移植后3个月内急性移植物抗宿主病（aGVHD）发生率为9.6%，而CRS≥3级组移植后3个月内未观察到aGVHD，但两组移植后3个月内aGVHD发生率差异无统计学意义（P=0.146）。haplo-HSCT后3个月内两组均未观察到移植相关死亡。
CRS≥3级的患儿中性粒细胞植入时间早，CRS临床表现严重且多样，haplo-HSCT后4天内糖皮质激素使用率高。对于这些患儿，移植后早期使用低剂量糖皮质激素可能减轻CRS反应，降低aGVHD发生率，从而给患儿带来更多益处。haplo-HSCT后的CRS对患儿预后无显著影响。
