总结起病时有肾脏增大表现患儿的急性淋巴细胞白血病（ALL）临床特点，并与起病时无肾脏增大表现的患儿进行临床资料及预后比较。

回顾性分析2010年至2019年北京大学第一医院儿科血液肿瘤专业病房（以下简称“我科”）收治的未经过任何治疗的初诊ALL患儿的临床资料及随访结果，将所有患儿根据初诊时治疗前的超声肾脏测量值分为初诊时有肾脏增大（肾脏超声长径≥同年龄儿童2倍标准差，肾脏增大起病组）及无肾脏增大表现（非肾脏增大起病组）2组并进行比较。

自2010年至2019年，我科收治确诊的初治ALL患儿规律治疗共69例，诊断时中位年龄4岁，起病时有肾脏增大共14例（20%），其中，单侧肾脏增大3例，双侧肾脏增大11例。中位随访时间112个月。与非肾脏增大起病组相比，肾脏增大起病组患儿年龄偏小［（79.9±48.0）月龄vs（37.1±21.9）月龄，P＜0.05］；2组在性别构成、起病距确诊的时间、危险度分层、起病时髓外白血病侵犯情况、形态学完全缓解时间、无事件生存（EFS）时间方面，差异均无统计学意义（P＞0.05）。尽管2组患儿的肌酐及尿素水平差异无统计学意义（P＞0.05），但考虑到2组患儿的年龄差异，认为肾脏增大起病组患儿的肾功能差于非肾脏增大起病组，但肾功能异常在治疗后均可恢复。E2A-PBX1融合基因阳性率在肾脏增大起病组患儿中占比高于非肾脏增大起病组（28.57% vs 5.36%，P＜0.05）。

初诊ALL患儿中起病时有肾脏增大表现的患儿比例高于既往文献报道，提示该现象较为普遍，起病时有肾脏增大的患儿年龄偏小。随着分层治疗的进一步优化，肾脏增大的患儿总体预后与起病时无肾脏增大的患儿相同，然而仍然要注意在初期治疗过程中注意肾功能的保护。

To summarize the clinical features of acute lymphoblast leukemia (ALL) with and without nephromegaly at the onset of the disease, and compare the clinical data and prognosis between them.

The clinical data and follow-up results of ALL children who were treated at the Pediatric Hematology and Oncology Ward of Peking University First Hospital from 2010 to 2019 were analyzed retrospectively. The children were divided into either a kidney enlargement group (kidney length ≥ 2 standard deviations from the mean of children of the same age) or a non-kidney enlargement group according to the kidney length measured by ultrasound before treatment at the initial diagnosis.

From 2010 to 2019, 69 patients were primarily diagnosed with ALL and treated at Peking University First Hospital. Their median age was 4 years old. Fourteen (20%) cases had renal enlargement at the onset of the disease, including three cases of unilateral enlargement and eleven cases of bilateral enlargement. Median follow-up time was 112 months. Compared with the non-kidney enlargement group, the age of the children with kidney enlargement was smaller [(79.9±48.0) months vs (37.1±21.9) months, P＜0.05]. There were no significant differences in the gender composition, time from onset to diagnosis, risk stratification, extramedullary invasion, time to morphological complete remission, and event-free survival (EFS) time (P＞0.05). Although there were no statistical differences in creatinine and urea levels between the two groups (P＞0.05), considering the difference in age between the two groups, the renal function of the children with renal enlargement was worse than that of the control group, but the renal function abnormality could recover after treatment. The positive rate of E2A-PbX1 was higher in the children of kidney enlargement group than that of non-kidney enlargement group (28.57% vs 5.36%, P＜0.05).

The proportion of children with renal enlargement at onset is higher than that reported in the previous literature, which suggests that the phenomenon is more common. The age of children with renal enlargement at onset is smaller. With the further optimization of layered treatment, the overall prognosis of children with renal enlargement is the same as that of children without kidney enlargement at the onset. However, we should pay attention to the protection of renal function in the initial treatment.