Huang Yu-Hua, Wu Qiu-Liang, Zong Yong-Sheng, Feng Yan-Fen, Liang Jian-Zhong, Hou Jing-Hui, Shao Qiong, Fu Jia
State Key Laboratory of Oncology in South China, Guangzhou, Guangdong, 510060, P.R. China.
Ai Zheng. 2009 Aug;28(8):805-12. doi: 10.5732/cjc.008.10847.
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.
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.
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 sIgM+/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 LMP1. There were no significant differences in morphology and immunophenotype between EBV-positive and EBV-negative cases.
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.
散发性伯基特淋巴瘤(sBL)较为罕见,在中国其与爱泼斯坦-巴尔病毒(EBV)的关系尚不清楚。本研究旨在调查EBV感染流行地区广州散发性伯基特淋巴瘤的临床表现、形态学特征、免疫表型及EBV感染状况。
回顾21例sBL患者的临床资料。进行一组免疫组织化学染色,并应用EBV编码的小RNA(EBERs)原位杂交来鉴定EBV感染。
2000年1月至2007年10月,中山大学肿瘤防治中心2416例非霍奇金淋巴瘤(NHL)患者中确诊21例(0.87%)sBL。男女比例为4.25(17/4)。中位年龄为23岁。21例患者中,19例(90.48%)有淋巴结受累;16例(76.19%)有多个部位受累;12例(57.14%)处于晚期(III/IV期)。15例接受化疗或手术加化疗的患者2年生存率为56.00%。20例呈现sBL的典型形态,1例为浆细胞样分化的sBL变异型。这21例sBL的主要免疫表型为sIgM+/CD20+/CD10+/Bcl-6+/Bcl-2-[或Bcl-6+(>95%)/Bcl-2+(<10%)]/TdT-/Ki-67+ 100%。在20例可检测的病例中,11例在少数(3%-20%)肿瘤细胞中显示CD5表达。10例(47.62%)p53过度表达。6例(28.57%)有EBV感染,伴有EBNA1和EBERs表达,但无LMP1表达。EBV阳性和阴性病例在形态学和免疫表型上无显著差异。
sBL在广州地区罕见主要见于男孩和青年男性。大多数患者有淋巴结受累,形态学和免疫表型与地方性伯基特淋巴瘤相似。I型EBV潜伏感染与28.57%的病例相关。