[伯基特淋巴瘤患儿的临床特征与预后：62例分析]
[Clinical features and prognosis of children with Burkitt's lymphoma: an analysis of 62 cases].
作者信息
Wang Ying-Chao, DU Wei-Chuang, Yin Chu-Yun, Gong Xue, Li Yuan-Fang
机构信息
Department of Children's Hematology and Oncology, First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China.
出版信息
Zhongguo Dang Dai Er Ke Za Zhi. 2022 May 15;24(5):561-565. doi: 10.7499/j.issn.1008-8830.2111064.
OBJECTIVES
To study the clinical features and chemotherapy response of Burkitt's lymphoma (BL) in children and the influence of rituximab on the prognosis of children with BL.
METHODS
A retrospective analysis was performed for the medical data of 62 children with BL, including clinical features, therapeutic efficacy, and prognostic factors. The Cox regression model was used to identify the factors associated with poor prognosis in children with BL. According to whether rituximab was used, the children with advanced (stage III/IV) BL were divided into two groups: chemotherapy plus rituximab and chemotherapy alone. The prognosis was compared between the two groups.
RESULTS
For these 62 children, the median age of onset was 5 years (range 1-14 years), and there were 58 boys (94%) and 4 girls (6%). The primary site was abdominal cavity in 41 children (66%), and head and neck in 16 children (26%). There were 1 child with stage I BL (2%), 8 with stage II BL (13%), 33 with stage III BL (53%), and 20 with stage IV BL (32%). The median follow-up time was 29 months, with progression/recurrence observed in 15 children (24%), and the 3-year overall survival (OS) rate and event-free survival (EFS) rate were 82.8%±5.2% and 77.3%±5.8%, respectively. For the children with stage III/IV BL, there was a significant difference in the 3-year the OS rate between the chemotherapy plus rituximab group (16 children) and the chemotherapy alone group (30 children) (93.3%±6.4% vs 65.6%±9.9%, =0.042), while there was no significant difference in the 3-year EFS rate between the two groups (86.2%±9.1% vs 61.8%±10.1%, >0.05). The Cox regression analysis showed that central nervous system involvement, lactate dehydrogenase >1 000 U/L, and early incomplete remission were the factors associated with poor prognosis (<0.05).
CONCLUSIONS
Chemotherapy combined with rituximab can improve the prognosis of children with stage III/IV BL. Central nervous system involvement, elevated lactate dehydrogenase level, and early incomplete remission may indicate a poor prognosis in children with BL.
目的
研究儿童伯基特淋巴瘤（BL）的临床特征、化疗反应以及利妥昔单抗对儿童BL预后的影响。
方法
对62例儿童BL的医疗数据进行回顾性分析，包括临床特征、治疗效果和预后因素。采用Cox回归模型确定与儿童BL预后不良相关的因素。根据是否使用利妥昔单抗，将晚期（III/IV期）BL患儿分为两组：化疗加利妥昔单抗组和单纯化疗组。比较两组的预后情况。
结果
这62例患儿的中位发病年龄为5岁（范围1 - 14岁），其中男孩58例（94%），女孩4例（6%）。原发部位在腹腔的患儿有41例（66%），头颈部有16例（26%）。I期BL患儿1例（2%），II期BL患儿8例（13%），III期BL患儿33例（53%），IV期BL患儿20例（32%）。中位随访时间为29个月，15例患儿（24%）出现病情进展/复发，3年总生存率（OS）和无事件生存率（EFS）分别为82.8%±5.2%和77.3%±5.8%。对于III/IV期BL患儿，化疗加利妥昔单抗组（16例）和单纯化疗组（30例）的3年OS率有显著差异（93.3%±6.4% vs 65.6%±9.9%，P = 0.042），而两组的3年EFS率无显著差异（86.2%±9.1% vs 61.8%±10.1%，P>0.05）。Cox回归分析显示，中枢神经系统受累、乳酸脱氢酶>1 000 U/L以及早期未完全缓解是与预后不良相关的因素（P<0.05）。
结论
化疗联合利妥昔单抗可改善III/IV期BL患儿的预后。中枢神经系统受累、乳酸脱氢酶水平升高以及早期未完全缓解可能提示儿童BL预后不良。
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