Objective: To investigate the clinical features and prognostic factors of acute lymphoblastic leukemia (ALL) children with P2RY8-CRLF2 gene rearrangement.
Methods: A total of 108 children with B-cell ALL (B-ALL) were diagnosed and systematically treated according to Chinese Children's Leukemia Group (CCLG) -ALL 2008 in our hospital from January 2016 to December 2016. The 108 patients were divided into two groups according to the result of mutiplex polymerase chain reaction: group with P2RY8-CRLF2 gene rearrangement and group without P2RY8-CRLF2 gene rearrangement. The ALL children with P2RY8-CRLF2 gene rearrangement were all treated by CCLG-ALL 2008 high-risk group (HR) regimens, and the ALL children in group without P2RY8-CRLF2 gene rearrangement received different intensity chemotherapy according to clinical risk classification.
Results: Five (4 male and 1 female) out of 108 patients with B-ALL had P2RY8-CRLF2 gene rearrangement. In the 5 B-ALL patients with P2RY8-CRLF2 gene rearrangement, the median age of the was 4 (2-6) years old and the median WBC count was 26.2 (2.46-525.1)×109/L. These patients presented different immunophenotype, including 3 cases of common B-ALL and 2 cases of pre B-ALL. Four patients carried a normal karyotype and 1 patient carried 46, XY, der (20) [22]/46, XY[2]. For the children with P2RY8-CRLF2 gene rearrangement, 1 patient (20%) could not achieve complete remission (CR), and minimal residual disease (MRD) of 2 patients (40%) was higher than 1% on day 33 of induction chemotherapy; while in group without P2RY8-CRLF2 gene rearrangement, all the patient achieved CR, and MRD in 6 patients (5.8%) was higher than 1% on day 33 of induction chemotherapy. The 3 year event-free survival (EFS) of ALL children in group with P2RY8-CRLF2 gene rearrangement was significantly lower than that in group without P2RY8-CRLF2 gene rearrangement (60.0%±21.9% vs 85.9%±3.9%) (P<0.05).
Conclusion: The early treatment response and prognosis of ALL children with P2RY8-CRLF2 gene rearrangement are worse, and more effective protocol is needed for this subtype patients.
题目: P2RY8-CRLF2重排的急性淋巴细胞白血病儿童的临床特征及预后分析.
目的: 探讨P2RY8-CRLF2重排急性淋巴细胞白血病（ALL）儿童的临床特征及预后.
方法: 2016年1-12月确诊并按照中国儿童白血病协作组（CCLG）-ALL 2008（CCGL-ALL 2008）方案规范治疗的108例B细胞型ALL（B-ALL）患儿，根据有无P2RY8-CRLF2重排分为P2RY8-CRLF2重排组和P2RY8-CRLF2阴性组。P2RY8-CRLF2重排组患儿均接受CCLG-ALL 2008高危组（HR）方案治疗，P2RY8-CRLF2阴性组患儿则按临床危险度分型接受不同强度化疗.
结果: 共检出P2RY8-CRLF2重排B-ALL患儿5例（4.6%），男4例，女1例，初诊中位年龄4（2-6） 岁，初诊白细胞计数中位数26.12（2.46-525.1）×109/L，3例为普通B细胞型，2例早期前B细胞型；1例46，XY，der(20) [22]/46，XY[2]，4例为正常核型。P2RY8-CRLF2重排组中，诱导缓解治疗第33天有1例（20%）未达完全缓解，2例（40%）微小残留病≥1%；P2RY8-CRLF2阴性组中，诱导缓解治疗第33天均达完全缓解率，6例（5.8%）微小残留病≥1%。P2RY8-CRLF2重排组3年无事件生存率显著低于P2RY8-CRLF2阴性组（60.0% ±21.9% vs 85.9%±3.9%） （P=<0.05）.
结论: 伴有P2RY8-CRLF2融合基因阳性的ALL儿童早期治疗反应较差，预后不良，仍需要探索更有效的治疗方法.