Paik Jin Ho, Choe Ji-Young, Kim Hyojin, Lee Jeong-Ok, Kang Hyoung Jin, Shin Hee Young, Lee Dong Soon, Heo Dae Seog, Kim Chul-Woo, Cho Kwang-Hyun, Kim Tae Min, Jeon Yoon Kyung
a Department of Pathology , Seoul National University Hospital, Seoul National University College of Medicine , Seoul , South Korea.
b Tumor Immunity Medical Research Center and Tumor Microenvironment Global Core Research Center, Seoul National University , Seoul , South Korea.
Leuk Lymphoma. 2017 Jan;58(1):53-63. doi: 10.1080/10428194.2016.1179297. Epub 2016 May 9.
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, 285 vs. 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.
爱泼斯坦-巴尔病毒阳性T/NK细胞淋巴增殖性疾病（EBV-T/NK-LPDs）包括几种重叠的与EBV相关的疾病，病程侵袭性各异。为了进行预后分类，我们回顾性分析了42例EBV-T/NK-LPD病例。男性（79%[33/42]）、年轻患者（≤40岁；83%[35/42]）以及T细胞谱系（81%[34/42]；CD8/CD4 = 1.8）占主导。临床病理方面，分为三个全身性类别和一个皮肤性类别：噬血细胞性淋巴组织细胞增生症（HLH；26%[11/42]）、慢性活动性EBV感染（CAEBV；31%[13/42]）、全身性无法分类疾病（24%[10/42]）以及种痘样水疱病/种痘样水疱病样淋巴瘤（HV/HVL；19%[8/42]）。预后方面，皮肤性疾病（HV/HVL）优于全身性疾病（p = 0.014；中位数，285个月对10个月）。在全身性疾病中，HLH最差（p = 0.002；3[HLH]对4[无法分类]对未达到[CAEBV]）。单因素生存分析（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）以及肝功能障碍（p = 0.023）预示生存期较短。多因素分析中，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。
