Age-related EBV-Associated B-Cell Lymphoproliferative disorders constitute a distinct clinicopathologic group: A study of 96 patients
Age-related EBV-Associated B-Cell Lymphoproliferative disorders constitute a distinct clinicopathologic group: A study of 96 patients
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DOI：
10.1158/1078-0432.ccr-06-2823
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发表时间：
2007-09-01
影响因子：
11.5
通讯作者：
Nakamura, Shigeo
中科院分区：
文献类型：
--
作者：
Oyama, Takashi;Yamamoto, Kazuhito;Nakamura, Shigeo
目的：我们最近报道了以老年患者为主的EBV+B细胞淋巴增生性疾病(EBV+B细胞增生性疾病)，这种疾病与免疫功能减退的患者虽然没有易感性免疫缺陷，但具有EBV+B细胞肿瘤的共同特征，被称为老年或与年龄相关的EBV+B细胞LPD。为了进一步明确这种疾病的特征，我们将年龄相关的EBV+B细胞LPD与EBV阴性的弥漫性大B细胞淋巴瘤(DLBCL)进行了比较。实验设计：在1,792例大B细胞LPD病例中，有可用的临床数据集的96例EBV+病例被纳入本研究。选择年龄在40岁以上的EBV阴性DLBCL患者107例作为对照组。结果：与EBV阴性的DLBCLs相比，年龄相关性EBV阳性的DLBCLs患者具有更高的年龄分布和侵袭性的临床特征或参数：44%的患者表现为功能状态，58%的患者血清乳酸脱氢酶水平高于正常，49%的患者有B症状，皮肤和肺的受累程度更高。因此，与年龄相关的EBV+组的总体存活率明显低于DLBCL组。单变量和多变量分析进一步确定了两个独立预测生存的因素，B症状和年龄超过70岁。使用这两个变量的预后模型很好地定义了三个风险组：低风险(无不利因素)、中风险(一个因素)和高风险(两个因素)。结论：这些发现表明与年龄相关的EBV+B细胞LPD构成了一个独特的组，应该为这种罕见的疾病开发创新的治疗策略，如EBV靶向T细胞治疗。
Purpose: We have recently reported EBV+ B-cell lymphoproliferative disorders (LPD) occurring predominantly in elderly patients, which shared features of EBV+ B-cell neoplasms arising in the immunologically deteriorated patients despite no predisposing immunodeficiency and were named as senile or age-related EBV+ B-cell LPDs. To further characterize this disease, age-related EBV+ B-cell LPDs were compared with EBV-negative diffuse large B-cell lymphomas (DLBCL).Experimental Design: Among 1,792 large B-cell LPD cases, 96 EBV+ cases with available clinical data set were enrolled for the present study. For the control group, 107 patients aged over 40 years with EBV-negative DLBCL were selected. We compared clinicopathologic data between two groups and determined prognostic factors by univariate and multivariate analysis.Results: Patients with age-related EBV+ B-cell LPDs showed a higher age distribution and aggressive clinical features or parameters than EBV-negative DLBCLs: 44% with performance status > 1, 58% with serum lactate dehydrogenase level higher than normal, 49% with B symptoms, and higher involvement of skin and lung. Overall survival was thus significantly inferior in age-related EBV+ group than in DLBCLs. Univariate and multivariate analyses further identified two factors, B symptoms and age older than 70 years, independently predictive for survival. A prognostic model using these two variables well defined three risk groups: low risk (no adverse factors), intermediate risk (one factor), and high risk (two factors).Conclusions: These findings suggest that age-related EBV+ B-cell LPDs constitute a distinct group, and innovative therapeutic strategies such as EBV-targetedT-cell therapy should be developed for this uncommon disease.