摘要
目的 探讨儿童伯基特淋巴瘤(Burkitt lymphoma,BL)的临床病理学特征及PCBP1表达意义.方法 收集47例儿童BL的临床资料,免疫组化检测PCBP1、MUM1、CD38、c-myc和LMO2,原位杂交检测EBER,荧光原位杂交检测c-myc、11q23.3/11q24.3基因.统计学分析PCBP1与临床参数及MUM1的表达关系.结果 47例患者中男性36例、女性11例,平均就诊年龄6岁2月(2岁1月～13岁),发病部位腹腔35例、头颈部11例、鼻腔1例;临床分期为Ⅱ期10 例、Ⅲ～Ⅳ期 37 例.免疫表型 PCBP1、MUM1、CD38、c-myc 和 LMO2 的阳性表达率分别为 100％(47/47)、63.8％(30/47)、100％(47/47)、97.9％(46/47)、0％(0/47);EBER 阳性率为 27.7％(13/47),c-myc 基因重排率为 100％(47/47),无11q23.3/11q24.3基因异常(0/15).47例BL中PCBP1细胞核和细胞质同时阳性表达38例,9例仅为细胞质阳性表达.PCBP1阳性表达模式与性别、发病年龄、发病部位、临床分期、MUM1和EBER表达均无关(P＞0.05).结论 儿童伯基特淋巴瘤主要与伴11q异常的高级别B细胞淋巴瘤、弥漫性大B细胞淋巴瘤进行鉴别,联合检测CD38、c-myc、LMO2和EBER具有诊断和鉴别诊断价值.PCBP1在儿童伯基特淋巴瘤中高表达,其表达模式的具体意义和机制有待进一步研究.
Abstract
Objective To investigate the clinical and pathological characteristics of Burkitt lymphoma(BL)in children and the significance of PCBP1 expression.Methods The clinical data of 47 children with BL were collected.PCBP1,MUM1,CD38,c-myc and LMO2 were detected by immunohistochemistry,EBER was detected by in situ hybridization,and c-myc,11q23.3/1 1q24.3 genes were detected by fluorescence in situ hybridization.Statistical analysis of the relationship between PCBP1 and clinical parameters,as well as the expression of MUM1.Results Among the 47 cases of BL,there were 36 males and 11 females,with an average age of 6 years and 2 months(2 years and 1 month to 13 years old).35 cases occurred in the abdomi-nal cavity,11 cases in the head and neck,and 1 case in the nasal cavity;The clinical staging was stage Ⅱ in 10 cases and stageⅢ～Ⅳ in 37 cases.The positive expression rates of immunophenotypes PCBP1,MUM1,CD38,c-myc,and LMO2 were 100％(47/47),63.8％(30/47),100％(47/47),97.9％(46/47),and 0％(0/47),respectively;The positive rate of EBER was 27.7％(13/47),the rearrangement rate of c-myc gene was 100％(47/47),and there was no 11 q23.3/11q24.3 gene abnormali-ty(0/15).Among 47 BL cases,38 cases showed simultaneous positive expression of PCBP1 in the nucleus and cytoplasm,while 9 cases showed only positive expression in the cytoplasm.The positive expression pattern of PCBP1 was not statistically significant with gender,age of onset,site of onset,clinical stage,MUM1 and EBER expression(P＞0.05).Conclusion Children's Burkitt's lymphoma is mainly differentiated from advanced B-cell lymphoma with 11 q abnormalities and diffuse large B-cell lymphoma.Combined detection of CD38,c-myc,LMO2,and EBER has diagnostic and differential diagnostic value.PCBP1 is highly expressed in pediatric Burkitt lymphoma,and the specific significance and mechanism of its expression pattern need further research.
基金项目
江西省卫生健康委员会科技计划项目(20203677)
江西省卫生健康委员会科技计划项目(202311080)
NETL
NSTL
万方数据