广州地区Burkitt’s淋巴瘤的临床病理特征及EB病毒感染状况(英文)

Clinicopathologic features and Epstein-Barr virus infection status of Burkitt's lymphoma in Guangzhou district

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 and Objective： Sporadic Burkitt＇s lymphoma （sBL） is uncommon and its relation to Epstein-Barr virus （EBV） is unknown in China. This study was to investigate the clinical presentation, morphologic features, immunophenotype and EBV infection status of sBL in Guangzhou district, a prevalent area of EBV infection. Methods： The clinical data of 21 sBL patients were reviewed. A panel of immunohistochemical staining was performed and EBV-encoded small RNAs （EBERs） in situ hybridization was applied to identify EBV infection. Results： From January 2000 to October 2007, 21 cases （0.87%） of sBL were confirmed among 2416 cases of non-Hodgkin＇s lymphoma （NHL） in Sun Yat-sen University Cancer Center. Male to female ratio was 4.25 （17/4）. The median age was 23 years. Of the 21 patients, 19 （90.48%） had lymph node（s） involvement; 16 （76.19%） had multiple sites involvement; 12 （57.14%） were at advanced stages （Ⅲ/Ⅳ ）. The 2-year survival rate of 15 patients who received chemotherapy or resection plus chemotherapy was 56.00%. Twenty cases showed the prototypic morphology of sBL, and one was the variant of sBL with plasmacytoid differentiation. The main immunophenotype of these 21 sBLs was slgM＋/CD20＋/CD10＋/Bcl- 6＋/Bcl-2-[or Bcl-6＋（〉95%）/Bcl-2^ ＋（〈10%） ]/TdT^-/Ki-67^＋ 100%. Of 20 detectable cases, 11 showed CD5 expression in a few （3%-20%） tumor cells. P53 was overexpressed in ten cases （47.62%）. Six cases （28.57%） had EBV infection, with EBNA1 and EBERs expression, but not LMPI. There were no significant differences in morphology and immunophenotype between EBV-positive and EBV-negative cases. Conclusions, sBL is uncommon in Guangzhou district, mainly seen in boys and young men. Most patients had lymph node （s） involvement, showing similar morphology and immunophenotype as that of endemic BL. Type I EBV latent infection is associated with 28.57% of cases.