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Primary central nervous system Epstein–Barr virus-positive diffuse large B-cell lymphoma of the elderly: a clinicopathologic study of five cases

机译:原发性中枢神经系统爱泼斯坦-巴尔病毒阳性的老年人弥漫性大B细胞淋巴瘤:5例临床病理研究

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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.
机译:我们报告了五例老年人的原发性中枢神经系统(CNS)爱泼斯坦-巴尔病毒(EBV)阳性淋巴瘤。这表示在134例EBV筛查后,原发性CNS弥漫性大B细胞淋巴瘤(DLBCL)的发生率为4%。所有五名患者均年满65岁或以上,且先前均无先天性或医源性免疫缺陷病史。所有病例的组织学形态均为DLBCL,坏死量可变。通过汉斯算法确定的起源细胞(COO)在2例中显示生发中心类型,在3例中显示非生发中心类型。未检测到MYC易位,仅在一种情况下检测到MYC过表达。诊断后不久有3例患者死亡,其余2例分别完全缓解2年和10年。我们得出结论,在美国东部原发性中枢神经系统淋巴瘤中,老年人中的EBV + DLBCL并不常见。患者通常表现为具有头痛和感觉运动症状的单一肿块病变。组织学形态为DLBCL,但可检测到克隆性T细胞基因重排。结果因案例而异,似乎与COO或MYC状态无关。

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