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年龄相关的EB病毒相关B细胞淋巴增殖性疾病构成一个独特的临床病理组:96例患者的研究

Age-related EBV-associated B-cell lymphoproliferative disorders constitute a distinct clinicopathologic group: a study of 96 patients.

作者信息

Oyama Takashi, Yamamoto Kazuhito, Asano Naoko, Oshiro Aya, Suzuki Ritsuro, Kagami Yoshitoyo, Morishima Yasuo, Takeuchi Kengo, Izumo Toshiyuki, Mori Shigeo, Ohshima Koichi, Suzumiya Junji, Nakamura Naoya, Abe Masafumi, Ichimura Koichi, Sato Yumiko, Yoshino Tadashi, Naoe Tomoki, Shimoyama Yoshie, Kamiya Yoshikazu, Kinoshita Tomohiro, Nakamura Shigeo

机构信息

Department of Clinical Oncology, Nagoya University Graduate School of Medicine, Nagoya, Japan.

出版信息

Clin Cancer Res. 2007 Sep 1;13(17):5124-32. doi: 10.1158/1078-0432.CCR-06-2823.

Abstract

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-targeted T-cell therapy should be developed for this uncommon disease.

摘要

目的

我们最近报道了主要发生在老年患者中的EBV阳性B细胞淋巴增殖性疾病(LPD),尽管没有易患免疫缺陷,但这些疾病具有免疫功能恶化患者中出现的EBV阳性B细胞肿瘤的特征,被命名为老年或年龄相关的EBV阳性B细胞LPD。为了进一步表征这种疾病,将年龄相关的EBV阳性B细胞LPD与EBV阴性弥漫性大B细胞淋巴瘤(DLBCL)进行了比较。

实验设计

在1792例大B细胞LPD病例中,96例有可用临床数据集的EBV阳性病例被纳入本研究。对于对照组,选择了107例年龄超过40岁的EBV阴性DLBCL患者。我们比较了两组之间的临床病理数据,并通过单因素和多因素分析确定了预后因素。

结果

与EBV阴性DLBCL相比,年龄相关的EBV阳性B细胞LPD患者表现出更高的年龄分布和侵袭性临床特征或参数:44%的患者体能状态>1,58%的患者血清乳酸脱氢酶水平高于正常,49%的患者有B症状,皮肤和肺部受累较多。因此,年龄相关的EBV阳性组的总生存期明显低于DLBCL组。单因素和多因素分析进一步确定了两个因素,即B症状和年龄大于70岁,可独立预测生存。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(无不良因素)、中风险(一个因素)和高风险(两个因素)。

结论

这些发现表明,年龄相关的EBV阳性B细胞LPD构成了一个独特的组,应该为这种罕见疾病开发如EBV靶向T细胞治疗等创新治疗策略。

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