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背景与目的:散发性Burkitt’s淋巴瘤(sporadic Burkitt’s lymphoma,sBL)少见,中国sBL的EB病毒(Epstein-Barrvirus,EBV)感染状况尚未见报道。本研究探讨广州地区sBL的临床特点、形态学特征、免疫表型及EBV的感染状况。方法:分析总结21例sBL患者的临床资料,并作一系列免疫组织化学染色确定免疫表型,EBV编码的小RNA(EBV-encoded small RNAs,EBERs)原位杂交检测EBV感染情况。结果:中山大学肿瘤防治中心2000年1月~2007年10月收治的2416例非霍奇金淋巴瘤中,明确诊断21例sBL(0.87%)。男女之比为4.25:1(17/4);中位年龄23岁。21例患者中,19例(90.48%)有淋巴结侵犯;16例(76.19%)多部位受累;12例(57.14%)临床分期为Ⅲ/Ⅳ期;15例化疗或手术加化疗患者的2年生存率为56.00%。20例形态学上表现为经典BL,另1例为伴有浆细胞样分化的BL变异型。该21例sBL主要的免疫表型为sIgM+/CD20+/CD10+/Bcl-6+/Bcl-2-[或Bcl-6+(>95%)/Bcl-2+(<10%)]/TdT-/Ki-67+100%。20例可检测病例中,11例有少数肿瘤细胞(3%~20%)表达CD5。10例肿瘤(47.62%)呈P53蛋白过表达。6例(28.57%)肿瘤细胞感染EBV,表达EBNA1和EBERs,但不表达LMP1。EBV阳性及阴性病例在形态学和免疫表型上均无显著差异。结论:广州地区sBL少见,主要见于儿童及年轻成年男性。多数病例有淋巴结的侵犯。形态学及免疫表型与地方性BL类同。28.57%病例呈EBVI型潜伏感染。
BACKGROUND & OBJECTIVE: Sporadic Burkitt’s lymphoma (sBL) is rare and Epstein-Barr virus (EBV) infection has not been reported in China. This study was to investigate the clinical features, morphological features, immunophenotype and EBV infection of sBL in Guangzhou. Methods: Clinical data of 21 patients with sBL were analyzed and analyzed. Immunohistochemistry and EBV-encoded small RNAs (EBERs) were used to detect EBV infection by immunohistochemical staining. Results: Twenty-one cases of sBL (0.87%) were correctly diagnosed in 2416 cases of non-Hodgkin’s lymphoma treated by Sun Yat-sen University Cancer Center from January 2000 to October 2007. The ratio of men to women was 4.25: 1 (17/4); the median age was 23 years. Of the 21 patients, 19 (90.48%) had lymph node involvement, 16 (76.19%) had multi-site involvement, 12 (57.14%) had clinical stage Ⅲ / Ⅳ, and 15 had chemotherapy or surgery plus chemotherapy for 2 years Survival rate was 56.00%. Twenty cases were morphologically characterized by classic BL and the other was BL variant with plasmacytoid differentiation. The main immunophenotypes of 21 patients with sBL were sIgM + / CD20 + / CD10 + / Bcl-6 + / Bcl-2- [or Bcl-6 + (> 95%) / Bcl-2 + / Ki-67 + 100%. Of the 20 detectable cases, 11 cases had a small number of tumor cells (3% -20%) expressing CD5. P53 protein was overexpressed in 10 of 10 cases (47.62%). Six cases (28.57%) of the tumor cells were infected with EBV, EBNA1 and EBERs were expressed, but LMP1 was not expressed. There was no significant difference in morphological and immunophenotype between EBV-positive and -negative cases. Conclusion: Guangzhou sBL rare, mainly seen in children and young adult men. Most cases have lymph node violations. Morphology and immunophenotypes are similar to endemic BL. 28.57% cases were EBVI latent infection.