儿童复发急性淋巴细胞白血病疗效及预后因素单中心分析

Efficacy and prognostic risk factors of childhood relapsed acute lymphoblastic leukemia：analysis from a single center

目的：探讨单中心复发急性淋巴细胞白血病（ALL）患儿采用ALL-R-2003方案的疗效和预后风险因素。方法回顾性分析2004年1月至2014年12月51例诊断为复发ALL患儿的临床资料，进行统计学分析。结果51例患儿中位初诊年龄5.5岁（0.8～13.4岁），中位复发时间为初诊后25个月（3～68个月），中位随访时间39个月（3～116个月）。复发患儿初发时标危、中危及高危组所占比例分别为27.5%（14／51）、29.4%（15／51）和43.1%（22／51）。复发后3年总生存（OS）率和无事件生存（EFS）率分别为（18.8±5.9）%和（16.2±5.8）%。非常早期复发、早期复发及晚期复发患儿的复发后3年OS率分别为0、（11.7±7.7）%和（51.7±14.8）%（P＝0.000）。不同复发部位和不同免疫表型患儿的复发后生存时间差异无统计学意义（P＞0.05）。按复发危险度分组的S1、S2、S3、S4组3年OS率分别为（50.0±35.4）%、（39.9±1.3）%、（10.0±9.5）%和0（P＝0.000）。 bcr-abl、MLL融合基因阳性者复发后3年OS率分别为（25.0±21.7）%和0，融合基因阴性者复发后3年OS率为（24.1±12.0）%，差异无统计学意义（P＞0.05）。复发后行骨髓移植组与未行移植组患儿的复发后3年OS率分别为（40.0±15.5）%和（13.0±5.9）%（P＝0.038）。结论复发ALL患儿初发时为高危分组及复发时间早时，则预后差。bcr-abl或MLL基因阳性患儿复发后预后差，骨髓移植可延长患儿生存时间。初发分组、复发时间及移植是影响预后的因素，其中复发时间和移植是影响预后的独立因素。

Objective To investigate the efficacy and prognostic risk factors of ALL-R-2003 protocol in the treatment of relapsed childhood relapsed acute lymphoblastic leukemia （ALL） in single center. Methods A retrospective study of clinical data of 51 children with relapsed ALL from January 2004 to December 2014 was performed by using SPSS version 19.0 statistical software for statistical analysis. Results The median age at initial diagnosis of 51 patients was 5.5 years （range, 0.8-13.4 years）. The median time from initial diagnosis to relapse was 25 months （range, 3-68 months） and follow-up time was 39 months （range, 3-116 months）. The relapse rate in the standard-risk, intermediate-risk and the high-risk groups were 27.5 % （14/51）, 29.4 %（15/51） and 43.1 % （22/51）, respectively. The probability of 3-year overall survival （pOS） after relapse was （18.8±5.9）%and the probability of event free survival （pEFS） was （16.2±5.8）%. The 3-year pOS in very early relapse, early relapse and late relapse were 0, （11.7 ±7.7） % and （51.7 ±14.8） %, respectively （P= 0.000）. There was no statistical difference in survival rate of different immunophenotype groups and sites of relapse （P〉 0.05）. The 3-year pOS of group S1, S2, S3, S4 were （50.0±35.4） %, （39.9±1.3） %, （10.0±9.5） % and 0, respectively （P=0.000）. The 3-year pOS of bcr-abl and MLL gene positive groups were （25.0±21.7） %and 0, respectively, with no statistically significance compared with the negtive group [（24.1±12.0）%] （P〉0.05）. The 3-year pOS rates of children with bone marrow transplantation and without transplantation were （40.0 ±15.5） %and （13.0 ±5.9） % respectively （P= 0.038）. Conclusions The children who in high risk group at initial diagnose are easily to meet earlier relapse and poorer prognosis. The survival period after relapse of bcr-abl or MLL gene positive cases is very short. Bone marrow transplantation can improve survival rate. Risk group at initial diagnose, relapse time and transplantation are the main factors influencing prognosis, and the relapse time and transplantation are the independent prognostic factors for relapsed childhood ALL.