Varying Presentations of EBV-associated Clinical Entities with Emphasis on EBV-Associated T- and NK-cell Lymphoproliferative Disorders of Childhood

doi:10.33696/pathology.2.028

Abstract

The Epstein-Barr Virus-associated T- and NK-cell lymphoproliferative disorders are a group of hematologic disturbances that occur in pediatric age groups. The literature describes extensive heterogeneity in the presentation of this disease, with a noncanonical reaction to Epstein-Barr virus infection underpinning the wide variety of clinical manifestations. In this review, we discuss chronic active Epstein-Barr virus infection, hypersensitivity to mosquito bites, Alice in Wonderland syndrome, Hydroa vacciniforme-like lymphoproliferative disorder, and the clinical and histologic findings of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis and systemic Epstein-Barr-positive T-cell lymphoproliferative disorder of childhood.

Highlights

The Epstein-BarrVirus (EBV) is an endemic human herpesvirus, with 90% of people demonstrating serological evidence of prior exposure to EBV
In a small percentage of cases, EpsteinBarr virus infection may manifest as a noncanonical reaction, yielding a wide variety of clinical manifestations
Immune system dysfunction is not characteristically associated with chronic active EBV infection (CAEBV) infection, though proliferating T- and NK-cells may infiltrate the parenchyma of various organs involved [4]

Summary

Introduction

The Epstein-BarrVirus (EBV) is an endemic human herpesvirus, with 90% of people demonstrating serological evidence of prior exposure to EBV. In a small percentage of cases, EpsteinBarr virus infection may manifest as a noncanonical reaction, yielding a wide variety of clinical manifestations. A rare but serious complication of primary EBV infection is the development of an EBV-associated lymphoproliferative complications, which can have devastating outcomes [1]. These entities are generally classified under the larger family of EBV-positive lymphoproliferative disorders [2]. This mini review describes the various lymphoproliferative disorders associated with EBV infection, outlining the pathophysiology, histology, and prognosis of each lymphoproliferative manifestation of EBV infection

Canonical EBV Exposure

Clinical Features

Histology and Immunohistochemistry

Findings

Differential Diagnosis