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Epstein-Barr virus (EBV)-positive sporadic burkitt lymphoma: an age-related lymphoproliferative disorder?

EB病毒(EBV)阳性的散发性伯基特淋巴瘤:一种年龄相关性淋巴组织增殖性疾病?

Satou A,Asano N,Nakazawa A,Osumi T,Tsurusawa M,Ishiguro A,Elsayed AA,Nakamura N,Ohshima K,Kinoshita T,Nakamura S

Abstract

Epstein-Barr virus (EBV) is detected in 20% to 30% of sporadic Burkitt lymphoma (sBL). However, only a few studies of EBV-positive (EBV) sBL have been reported, and its characteristics still remain controversial. To highlight the features of EBV sBL, we compared the clinicopathologic characteristics of 33 cases of EBV and 117 cases of EBV-negative (EBV) sBL in Japan. EBV sBL showed significantly higher age distribution (median, 42 vs. 13 y; P<0.0001) and higher frequency of patients older than 50 years (48% vs. 16%, P<0.0001). We also revealed the difference of the involved sites. The EBV group showed significantly higher incidence of involvement of tonsil (P=0.027), adrenal gland (P=0.011), and cervical lymph node (P=0.040). In addition, the EBV group tended to have higher incidence of nodal involvement (P=0.078) and involvement of para-aorta lymph node (P=0.084) and heart (P=0.050). In contrast, the gastrointestinal tract was less frequently affected in EBV sBL (P=0.024). In addition, the less positivity for MUM1 (P=0.020) of EBV sBL was highlighted. These results indicate that biological behavior and pathogenesis of EBV sBL might be different from those of EBV sBL. Our results demonstrate that EBV sBL has an aspect of age-related disease and is a distinct clinicopathologic subtype, which should be distinguished from EBV sBL.

摘要

20%-30%的散发性伯基特淋巴瘤(sBL)检测出EB病毒(EBV)。然而,仅少数EBV阳性sBL的病例报道,其特征仍存在争议。为了突出EBV性sBL的特征,我们对日本33例EBV阳性和117例EBV阴性sBL的临床病理特征进行了比较。EBV阳性sBL显示出明显更高的年龄分布(中位年龄42岁vs13岁;P<0.0001),以及大于50岁患者发病率更高(48%vs16%,P<0.0001)。我们还发现累及部位的差异。EBV组累及扁桃体(P=0.027)、肾上腺(P=0.011)和颈部淋巴结(P=0.040)的发生率明显更高。此外,EBV组倾向累及淋巴结 (P=0.078)、主动脉旁淋巴结(P=0.084)及心脏(P=0.050)。相反,胃肠道不常受EBV sBL的影响(P=0.024)。另外,EBV sBL的MUM1阳性表达减少(P=0.020)也非常显著。这些结果显示EBV sBL的生物学行为和发病机理可能不同于EBV阴性sBL。我们的结果表明EBV sBL是一种年龄相关性疾病,其作为一种独特的临床病理亚型应与EBV阴性sBL相鉴别。

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