Primary central nervous system Epstein-Barr virus-positive diffuse large B-cell lymphoma of the elderly: a clinicopathologic study of five cases
Primary central nervous system Epstein-Barr virus-positive diffuse large B-cell lymphoma of the elderly: a clinicopathologic study of five cases
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DOI：
10.1007/s10014-013-0173-x
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发表时间：
2014-10-01
影响因子：
3.3
通讯作者：
Gong, Jerald Z.
中科院分区：
文献类型：
--
作者：
El Jamal, Siraj;Li, Shiyong;Gong, Jerald Z.
我们报告五例老年原发性中枢神经系统EB病毒(EBV)阳性淋巴瘤。134例初发中枢神经系统弥漫性大B细胞淋巴瘤(DLBCL)经EBV筛查后，发病率为4%。所有五名患者年龄都在65岁或以上，以前没有先天性或医源性免疫缺陷的病史。所有病例的组织学形态均为DLBCL，可见不同程度的坏死灶。Hans算法确定的原始细胞(COO)为生发中心型2例，非生发中心型3例。未检测到MYC易位，仅1例MYC过表达。3名患者在确诊后不久死亡，其余2名患者分别完全缓解2年和10年。我们得出的结论是EBV？老年人DLBCL在美国东部的原发中枢神经系统淋巴瘤中并不常见。患者通常表现为单发肿块，伴有头痛和感觉运动症状。组织学形态为DLBCL，但可检测到克隆性T细胞基因重排。结果因个案而异，似乎与COO或MYC的地位无关。
We report five cases of primary central nervous system (CNS) Epstein-Barr virus (EBV)-positive lymphoma of the elderly. This represented an incidence of 4 % of primary CNS diffuse large B-cell lymphoma (DLBCL) after EBV screening in 134 cases. All five patients were 65 years or older with no previous history of congenital or iatrogenic immune deficiencies. The histologic morphology of all the cases was DLBCL, with variable amounts of necrosis. The cell of origin (COO) as determined by the Hans algorithm disclosed germinal center type in 2 cases and non-germinal center type in 3 cases. MYC translocation was not detected, and MYC overexpression was detected in only one case. Three patients died shortly after diagnosis, and the remaining 2 patients were in complete remission for 2 and 10 years, respectively. We conclude that EBV? DLBCL among the elderly is uncommon in primary CNS lymphoma in the Eastern United States. The patients usually present with a single mass lesion with headache and sensorimotor symptoms. The histologic morphology is DLBCL, but clonal T-cell gene rearrangement may be detected. The outcome varies from case to case and appears to be unrelated to the COO or MYC status.