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Background  Systemic Epstein-Barr virus +  T-cell lymphoma (sEBV +  TCL) occurs in childhood and young adults, and is exceptionally rare in older adults.    Methods  We investigated clinicopathological features in 16 patients of various ages with systemic EBV +  CD8 +  T-lymphoproliferative diseases.    Results  Eight 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).    Conclusion  sEBV +  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.

Clinicopathological findings of systemic Epstein-Barr virus-positive T-lymphoproliferative diseases in younger and older adults