Clinicopathological categorization of Epstein–Barr virus-positive T/NK-cell lymphoproliferative disease: an analysis of 42 cases with an emphasis on prognostic implications

Jin Ho PaikDepartment of Pathology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, South Korea; ;Tumor Immunity Medical Research Center and Tumor Microenvironment Global Core Research Center, Seoul National University, Seoul, South Korea; ;Department of Pathology, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, South Korea;

Ji-Young ChoeDepartment of Pathology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, South Korea; ;Department of Pathology, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, South Korea;

Hyojin KimDepartment of Pathology, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, South Korea;

Jeong-Ok LeeDepartment of Internal Medicine, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, South Korea;

Hyoung Jin KangDepartment of Pediatrics, Cancer Research Institute, Seoul National University College of Medicine, Seoul National University Children’s Hospital, Seoul, South Korea;

Hee Young ShinDepartment of Pediatrics, Cancer Research Institute, Seoul National University College of Medicine, Seoul National University Children’s Hospital, Seoul, South Korea;

Dong Soon LeeDepartment of Laboratory Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, South Korea;

Dae Seog HeoDepartment of Internal Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, South Korea;

Chul-Woo KimDepartment of Pathology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, South Korea; ;Tumor Immunity Medical Research Center and Tumor Microenvironment Global Core Research Center, Seoul National University, Seoul, South Korea;

Kwang-Hyun ChoDepartment of Dermatology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, South Korea

Tae Min KimDepartment of Internal Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, South Korea; Correspondence[email protected] [email protected]

Yoon Kyung JeonDepartment of Pathology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, South Korea; ;Tumor Immunity Medical Research Center and Tumor Microenvironment Global Core Research Center, Seoul National University, Seoul, South Korea; Correspondence[email protected] [email protected]

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Abstract

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.

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Disclosure forms provided by the authors are available with the full text of this article at http://dx.doi.org/10.1080/10428194.2016.1179297.

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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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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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