【摘要】 目的：分析儿童急性淋巴细胞白血病（acute lymphoblastic leukemia,ALL）治疗过程中发生门冬酰胺酶（asparaginase,ASP）相关胰腺炎（asparaginase-associated pancreatitis,AAP）的临床特点以及影响AAP的危险因素，探讨AAP后ASP再暴露情况及AAP对ALL患儿预后的影响。方法：回顾性收集2015年1月至2020年6月于华中科技大学同济医学院附属同济医院初诊252例ALL患儿临床资料，分析AAP临床特点。使用Logistic回归分析AAP的危险因素，预后分析采用Log-rank检验，使用KaplanMeier法绘制生存曲线。结果：252例ALL患儿中，23例AAP发生率为9.1%。19例（82.6%）患儿AAP发生在诱导缓解期，末次ASP至胰腺炎发生的中位时间为12 d。初诊时总胆固醇≥3.5 mmol/L是AAP发生的独立危险因素（P<0.001）。6例AAP患儿在AAP治愈后进行ASP再暴露，3例发生二次AAP。AAP组患儿5年总体生存（overall survival,OS）为（78.3±8.6）%，非AAP组患儿5年OS为（90.3±2.2）%，差异有统计学意义（P<0.05）。中止组患儿5年OS为（77.8±9.8）%，未中止组患儿5年OS为（90.1±2.1）%，差异具有统计学意义（P<0.05）。结论：末次ASP至胰腺炎发生的中位时间为12 d。AAP患儿较非AAP患儿5年OS低。ASP再暴露有发生二次AAP的风险，但完成化疗方案ASP有助于改善预后。更多还原

【Abstract】 Objective: To analyze the clinical characteristics and risk factors of asparaginase-associated pancreatitis(AAP) in children with acute lymphoblastic leukemia(ALL), and to investigate the impact of AAP on their prognosis following re-exposure to asparaginase(ASP).Methods: Clinical children data with ALL at Tongji Hospital, Tongji Medical College of Huazhong University of Science and Technology between January 2015 and June 2020 were collected to analyze the clinical features of AAP. Logistic regression was used to identify risk factors for AAP. Prognostic analysis was performed using the Log-rank test and Kaplan–Meier survival curves. Results: Overall, 252 children with ALL were included, among whom 23(9.1%) developed AAP. Most AAP cases(82.6%) occurred during remission induction, with a median time from the last ASP to AAP of 12 d. Elevated total cholesterol(≥3.5 mmol/L) at initial diagnosis was identified as an independent risk factor. Six children(26.1%) were re-exposed to ASP, leading to recurrent pancreatitis in 3 cases. The 5-year overall survival(OS) was significantly lower in the AAP group(78.3%±8.6%) compared to the non-AAP group(90.3%±2.2%)(P<0.05). Similarly, children who discontinued ASP due to AAP had a 5-year OS of 77.8%±9.8%, significantly lower than the control group(90.1%±2.1%). Conclusions: AAP typically occurred within 12 d of the last ASP administration and was associated with poorer 5-year OS. Re-exposure to ASP posed a risk of recurrent AAP; however, completing the ASP chemotherapy regimen may be crucial for improving prognosis.更多还原