EBV-associated T/NK–cell lymphoproliferative diseases in nonimmunocompromised hosts: prospective analysis of 108 cases

Abstract

EBV-associated T/NK-cell lymphoproliferative disease (T/NK-LPD) is defined as

The aggressive peripheral T‐cell lymphomas: 2015

“…A recent report from Japan described EBVassociated T/NK-cell lymphoproliferative diseases that were not malignant but sometimes evolved into lymphoma [34]. Some patients had progressive EBV-associated hemophagocytic lymphohistocytosis, and 44% of patients died from organ complications.…”

Epstein-Barr Virus: A Spectrum of Disease from Mononucleosis to Lymphoma

“…Another fascinating aspect of the many links between the human immune system and EBV is the occurrence of chronic active EBV infections most common in Japan but also seen sporadically in other geographical areas (10,11). These infections often occur in childhood and are characterized by the atypical infection of T and NK lymphocytes and may culminate in lymphoma.…”

Defining the elusive boundaries of chronic active Epstein-Barr virus infection

“…EBV is also implicated in the development of several hematolymphoid malignancies derived from B-, T-and natural killer (NK)-cells, including Burkitt lymphoma, classic Hodgkin lymphoma, extranodal NK-/T-cell lymphoma, nasal type (ENKTL), and immunodeficiency-associated lymphoproliferative disorders (LPD). [1][2][3][4][5] Primarily because of their rarity, systemic EBV + T-cell and NK-cell LPDs are not completely understood. Criteria to separate clinical risk groups at diagnosis are confounded by several important factors: 1) non-specific histopathological and immunophenotypic features that overlap with infectious and inflammatory conditions as well as with other T-and NK-LPDs; 2) lack of markers to reliably predict clinical behavior that ranges from self-limiting proliferations to those that are rapidly fatal; 3) molecular clonality, although often detected, is not necessarily indicative of malignancy; and 4) inconsistent association with hemophagocytic lymphohistiocytosis (HLH), whose presence is often life-threatening.…”

“…The clinical course and prognosis of CAEBV is highly variable: while some patients exhibit a protracted disease course, others experience a fulminant form of the disease accompanied by HLH. [1][2][3][8][9][10] Chronic active EBV of T-and NK-cell types have a strong ethnic predisposition, and are most frequent in East Asia and in indigenous populations of Central and South America. 1,[6][7][8][9][10][11] It is rare in Western and African populations.…”

“…Criteria to separate clinical risk groups at diagnosis are confounded by several important factors: 1) non-specific histopathological and immunophenotypic features that overlap with infectious and inflammatory conditions as well as with other T-and NK-LPDs; 2) lack of markers to reliably predict clinical behavior that ranges from self-limiting proliferations to those that are rapidly fatal; 3) molecular clonality, although often detected, is not necessarily indicative of malignancy; and 4) inconsistent association with hemophagocytic lymphohistiocytosis (HLH), whose presence is often life-threatening. [1][2][3][6][7][8][9][10][11] Large, clinically well-annotated cohorts of patients offer a tremendous opportunity to investigate rare subtypes of EBV + T-and NK-cell proliferations, define diagnostic and prognostic criteria, and explore disease boundaries. To that end, the paper by Kawamoto et al in this issue of Haematologica addresses a rare type of EBV + T-and NK-cell proliferation in adult patients with chronic active EBV-like features (adult-onset CAEBV).…”