Clinicopathological categorization of Epstein-Barr virus-positive T/NK-cell lymphoproliferative disease: an analysis of 42 cases with an emphasis on prognostic implications
Clinicopathological categorization of Epstein-Barr virus-positive T/NK-cell lymphoproliferative disease: an analysis of 42 cases with an emphasis on prognostic implications
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DOI：
10.1080/10428194.2016.1179297
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发表时间：
2017-01-01
影响因子：
2.6
通讯作者：
Jeon，Yoon Kyung
中科院分区：
文献类型：
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作者：
Paik，Jin Ho;Choe，Ji-Young;Jeon，Yoon Kyung
Epstein-Barr 病毒阳性 T/NK 细胞淋巴增殖性疾病 (EBV-T/NK-LPD) 包括几种重叠的 EBV 相关病症，且具有不同的侵袭性病程。对于预后分类，我们回顾性分析了 42 例 EBV-T/NK-LPD 病例。男性（79% [33/42]）、年轻（≤40 岁；83% [35/42]）患者和 T 细胞谱系（81% [34/42]；CD8/CD4 = 1.8）占主导地位。临床病理学上，开发了三种系统性和一种皮肤性类别：噬血细胞性淋巴组织细胞增多症（HLH；26% [11/42]）、慢性活动性 EBV 感染（CAEBV；31% [13/42]）、系统性无法分类疾病（24% [10/42]）和痘痘水痘/痘痘水痘样淋巴瘤（HV/HVL； 19% [8/42]）。从预后来看，皮肤疾病 (HV/HVL) 优于全身性疾病（p= 0.014；中位数为 285 个月与 10 个月）。在全身性疾病中，HLH 最差（p= 0.002；3[HLH]vs.4[无法分类]vs.未达到[CAEBV]）。单变量生存分析 (n= 42) 显示血细胞减少（≥一个谱系；p< 0.001）、发病年龄（>40 岁；p= 0.001）、T 细胞谱系 (p= 0.041)、噬血细胞组织细胞 (p= 0.031)、乳酸脱氢酶升高(p= 0.020) 和肝功能障碍 (p= 0.023) 预测生存期较短。在多变量分析中，T 细胞谱系 (p= 0.025 [HR =11.3]) 和血细胞减少 (p= 0.028 [HR =5.4]) 是独立的预后因素。因此，EBV-T/NK-LPD可分为四个预后类别。
Epstein–Barr virus-positive T/NK-cell lymphoproliferative diseases (EBV-T/NK-LPDs) include several overlapping EBV-related conditions with variably aggressive courses. For prognostic categorization, we retrospectively analyzed 42 EBV-T/NK-LPD cases. Male (79% [33/42]), young (≤40 years; 83% [35/42]) patients and T-cell lineage (81% [34/42]; CD8/CD4 = 1.8) were predominant. Clinicopathologically, three systemic and one cutaneous category were developed: hemophagocytic lymphohistiocytosis (HLH; 26% [11/42]), chronic active EBV infection (CAEBV; 31% [13/42]), systemic unclassifiable disease (24% [10/42]), and hydroa vacciniforme/hydroa vacciniforme-like lymphoma (HV/HVL; 19% [8/42]). Prognostically, cutaneous disease (HV/HVL) was better than systemic disease (p= 0.014; median, 285vs.10 months). In systemic diseases, HLH was worst (p= 0.002; 3[HLH]vs.4[unclassifiable]vs.not reached [CAEBV]). Univariate survival analysis (n= 42) revealed cytopenia (≥one lineage;p< 0.001), onset age (>40 years;p= 0.001), T-cell lineage (p= 0.041), hemophagocytic histiocytes (p= 0.031), elevated lactate dehydrogenase (p= 0.020), and liver dysfunction (p= 0.023) predicted shorter survival. In multivariate analysis, T-cell lineage (p= 0.025 [HR =11.3]) and cytopenia (p= 0.028 [HR =5.4]) were independent prognostic factors. Therefore, EBV-T/NK-LPD could be classified into four prognostic categories.