Abstract
BackgroundSystemic Epstein-Barr virus+ T-cell lymphoma (sEBV+ TCL) occurs in childhood and young adults, and is exceptionally rare in older adults.MethodsWe investigated clinicopathological features in 16 patients of various ages with systemic EBV+ CD8+ T-lymphoproliferative diseases.ResultsEight younger patients and four of eight older adults had sEBV+ CD8+ TCL, with invasion by medium-sized to/or large atypical lymphocytes primarily in bone marrow and lymph nodes, hemophagocytic lymphohistiocytosis (HLH), and progressive clinicopathological course. A further two patients demonstrated EBV+ node-based CD8+ large TCL without HLH, while the remaining two had the systemic form of chronic active EBV infection (sCAEBV) with CD8+ small lymphocytes. Past history of sCAEBV-like lesions was observed in one sEBV+ TCL patient (8.3%). Immunohistologically, in 12 sEBV+ TCL patients, atypical lymphocytes were positive for phosphate signal transducer and activator of transcription 3 (66.7%), CMYC (83.3%), and p53 (75%). Strong reactions of programmed cell death-ligand (PD-L)1+ tumor or non-neoplastic cells were detected in nine sEBV+ TCL patients (75%). Clonal peaks of the T-cell receptor (TCR) γ gene were detected in eight sEBV+ TCL patients by polymerase chain reaction. Four younger patients in sEBV+ TCL (33.3%) are in remission with chemotherapies including etoposide, and three of the four underwent allogeneic stem cell transplantation (SCT).ConclusionsEBV+ CD8+ TCL was observed in younger and older adults with less history of sCAEBV. HLH, tumor cell atypia, immunohistological findings, and progressive clinical course were characteristic of sEBV+ CD8+ TCL. Prompt chemotherapy and SCT induced tumor regression in sEBV+ CD8+ TCL patients.
Highlights
Systemic Epstein-Barr virus+ T-cell lymphoma occurs in childhood and young adults, and is exceptionally rare in older adults
Epstein-Barr virus (EBV)+ nodal cytotoxic T-cell lymphoma (TCL) shows a primarily nodal presentation with limited extranodal disease and no nasal invasion, mainly demonstrating CD8+ and CD56-negative phenotype [11, 12]. systemic form of chronic active EBV infection (sCAEBV) presents mainly with infectious mononucleosis (IM)-like symptoms persisting for 3 months, elevated peripheral blood EBV DNA (≥ 1 × 102.5 copies/μg) and the presence of EBV+ T-lymphocytes with mild nuclear atypia [9, 14]
The median age of the 12 sEBV+ TCL patients was 36.1 years, four of whom were over 60 years of age
Summary
Systemic Epstein-Barr virus+ T-cell lymphoma (sEBV+ TCL) occurs in childhood and young adults, and is exceptionally rare in older adults. Epstein-Barr virus (EBV)-positive T/natural killer (NK)cell lymphoproliferative diseases (EBV+ TNKLPDs) with bone marrow involvement include aggressive NK-cell leukemia, hemophagocytic lymphohistiocytosis (HLH), and systemic EBV+ T-cell lymphoma (sEBV+ TCL) of childhood [1,2,3]. Patients with these TNKLPDs exhibit similar clinicopathological features of high fever, hepatosplenomegaly, pan- or bicytopenia, and coagulopathy. Atypical T-cells in bone marrow, lymph nodes, liver, and spleen, and patients demonstrate above described clinical features of TNKLPDs and mostly follow a rapidly progressive clinical course.
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