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多中心儿童伯基特白血病的临床特征及预后因素分析

英文原题:Analysis of the clinical characteristics and prognostic factors of multicenter childhood Burkitt leukemia.

查看英文原题

Analysis of the clinical characteristics and prognostic factors of multicenter childhood Burkitt leukemia.

PubMed 2025/11/03(内容时间) Ann Hematol Q3 · IF 2.3(JCR 2025)

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中文摘要

伯基特淋巴瘤(BL)是一种倍增时间快的B细胞恶性肿瘤,起源于滤泡生发中心。我们旨在探讨儿童伯基特白血病的特征及预后。共纳入124例在CNCL-B-NHL-2017方案(中国儿童淋巴瘤协作组成熟B细胞淋巴瘤2017方案)6年期间入组的伯基特白血病患儿进行评估。中位发病年龄为7岁(1-15岁),男女比例为4.17:1。其中,50.8%的患儿年龄为5-10岁。伯基特白血病患儿在诊断时更易出现肿瘤溶解综合征、肾功能不全、乳酸脱氢酶(LDH)>4倍、多器官受累及中枢神经系统浸润,而大肿瘤负荷者少见。11例患儿为单纯伯基特白血病(8.9%),与伴有肿瘤包块的患儿相比,其在复发、进展、初诊时白细胞计数、LDH水平、CNS浸润及治疗前肿瘤溶解综合征发生率方面均无显著差异(P > 0.05)。全组中位随访时间为32.85个月(0.4-70.7),3年总生存率和无事件生存率分别为87.1%和81.5%。13例(10%)患儿在治疗期间进展或复发,其中9例接受了CAR-T 细胞治疗,仅3例死亡。分析确定中期评估时残留病灶(P = 0.024)和LDH升高≥ 2000 U/L(P = 0.014)为影响生存的独立预后因素。CNCL-B-NHL-2017方案在治疗儿童伯基特白血病方面显示出显著疗效。

展开英文摘要原文

Burkitt lymphoma (BL) is a B-cell malignancy with a rapid doubling time, originating in follicular germinal centers.

We aimed to explore the characteristics and prognosis of childhood Burkitt leukemia. A total of 124 children with Burkitt leukemia enrolled during the 6-year period of China Net Childhood Lymphoma- mature B-cell lymphoma 2017 regimen (CNCL-B-NHL-2017) were assessed. The median age at onset was 7 years (1-15 years), with a male-to-female ratio of 4. 17:1. Of the total, 50. 8% children were aged 5-10 years. Children with Burkitt leukemia were more likely to have tumor lysis syndrome, renal insufficiency, lactate dehydrogenase (LDH) > 4 times, multiple organ involvement, and central nervous system infiltration at diagnosis, whereas those with large tumor mass were rare. Eleven children had pure Burkitt leukemia (8.

9%), with no significant differences in relapse, progression, white blood cell counts at initial diagnosis, LDH levels, CNS infiltration, and rates of tumor lysis syndrome before treatment compared to those of children with tumor masses. (P > 0. 05). The median follow-up time for the entire group was 32. 85 months (0. 4-70. 7), with 3-year overall survival and event-free survival rates of 87. 1% and 81. 5%, respectively.

Thirteen (10%) children progressed or relapsed during treatment, of which nine received chimeric antigen receptor T-cell therapy, with only three fatalities. The analysis identified residual disease at midterm evaluation (P = 0. 024) and LDH elevation ≥ 2000 U/L (P = 0. 014) as independent prognostic factors affecting survival. The CNCL-B-NHL-2017 protocol demonstrated significant efficacy in treating children with Burkitt leukemia.

论文信息

作者
Pei W、Duan Y、Jin L、Yang X、Dai Y、Zheng M、Zhang L、Liu Y
第一作者单位
Children's Hospital Affiliated to Shandong University (Jinan Children's Hospital), Jinan, 250022, China.China
通讯作者单位
Children's Hospital Affiliated to Shandong University (Jinan Children's Hospital), Jinan, 250022, China. lifusd@126.com.China
文献类型
多中心研究
期刊
Annals of hematology2025 Nov
原文标识
PubMed 41182358 · DOI 10.1007/s00277-025-06672-9