Department of Medicine I, Medical University of Vienna, Austria.
Pathology. 2010;42(6):547-52. doi: 10.3109/00313025.2010.508786.
Pathological and clinical data in a large series of immunocompetent patients with primary lymphoma of the central nervous system (PCNSL) were analysed.
We immunostained tumour specimens of 75 patients for CD3, CD4, CD5, CD8, CD10, CD20, CD30, CD79a, Bcl-2, Bcl-6, CD138, MUM1, TDT, PAX5, FOXP1 and Ki-67 and performed in situ hybridisation for Epstein-Barr virus (EBV) RNA. Eleven cases were investigated for rearrangements of BCL6, immunoglobulin heavy chain (IGH) and FOXP1 genes using fluorescent in situ hybridisation (FISH).
Histologically, most cases were classified as diffuse large B-cell lymphoma (80.2%) predominantly of centroblastic type. Immunophenotypic profiling revealed that 96% and 4% of cases corresponded to non-germinal centre and germinal centre type, respectively. FISH analysis showed t(3;14)/IGH-BCL6 in 2/11 cases and trisomy 3 in 2/11 cases. FOXP1 rearrangements were not found. At survival analysis, Karnofsky index >80 and presence of Bcl-6 expression showed independent significant association with favourable patient outcome.
PCNSL represents a histologically and immunophenotypically very homogeneous lymphoma type, probably derived from germinal centre exit B cells. The frequent overexpression of FOXP1 appears not to be related to FOXP1 gene rearrangement. Survival analyses disclosed Bcl-6 expression and high Karnofsky performance score as independent prognostic parameters associated with favourable outcome.
分析大量免疫功能正常的原发性中枢神经系统淋巴瘤（PCNSL）患者的病理和临床数据。
我们对 75 例患者的肿瘤标本进行了 CD3、CD4、CD5、CD8、CD10、CD20、CD30、CD79a、Bcl-2、Bcl-6、CD138、MUM1、TDT、PAX5、FOXP1 和 Ki-67 的免疫组化染色，并进行了 EBV RNA 的原位杂交。对 11 例病例进行了 BCL6、免疫球蛋白重链（IGH）和 FOXP1 基因重排的荧光原位杂交（FISH）检测。
组织学上，大多数病例被分类为弥漫性大 B 细胞淋巴瘤（80.2%），主要为中心母细胞型。免疫表型分析显示，96%和 4%的病例分别对应于非生发中心和生发中心型。FISH 分析显示，2/11 例存在 t（3;14）/IGH-BCL6，2/11 例存在三体 3。未发现 FOXP1 重排。在生存分析中，Karnofsky 指数>80 和 Bcl-6 表达阳性与患者预后良好有独立的显著相关性。
PCNSL 是一种组织学和免疫表型非常同质的淋巴瘤类型，可能来源于生发中心 B 细胞。FOXP1 的频繁过表达似乎与 FOXP1 基因重排无关。生存分析显示 Bcl-6 表达和高 Karnofsky 表现评分是与良好预后相关的独立预后参数。
