Table. 1.

Table. 1.

Classification of EBV-positive lymphoproliferative disorders

Diagnosis Main age group Pathogenesis EBV infected cell Clinical course
Chronic active EBV infection, B, systemic Children and young adults Genetic defect in host immune response to EBV B-cells Variable from indolent to aggressive
Chronic active EBV infection, T & NK, systemic Children and young adults Genetic defect in host immune response to EBV T or NK cells Variable from indolent to aggressive
Severe mosquito-bite allergy Children and young adults Genetic defect in host immune response to EBV Mainly NK cells Variable from indolent to aggressive
Hydroa-vacciniforme-like LPD Children and young adults Genetic defect in host immune response to EBV T cells Variable from indolent to aggressive
Systemic EBV-positive T cell lymphoma of childhood Children Genetic defect in host immune response to EBV T cells Fulminant
EBV-positive DLBCL, NOS Elderly and young adults Age-related immune deficiency in the elderly B-cells Depends on age; aggressive in the elderly
EBV-positive mucocutaneous ulcer Elderly Iatrogenic or age-related immune deficiency B-cells Mostly benign
DLBCL associated with chronic inflammation Adults Local immunodeficiency in a closed space B-cells Aggressive
Fibrin-associated DLBCL Adults Local immunodeficiency in a closed space B-cells Mostly benign
Lymphomatoid granulomatosis Adults Underlying immunodeficiency B-cells Variable depending on grade
Extranodal NK/T cell lymphoma, nasal-type Adults Oncogenic potential of EBV NK or T cells Aggressive
Aggressive NK cell leukemia Adults Oncogenic potential of EBV NK cells Fulminant
PTCL, NOS, EBV-positive Adults Immune deficiency T cells Aggressive
Oncogenic potential of EBV

LPD, lymphoproliferative disorders; DLBCL, diffuse large B cell lymphoma; PTCL, peripheral T cell lymphoma; NOS, not otherwise specified.

Clin Pediatr Hematol Oncol 2021;28:14-27
© 2021 Clin Pediatr Hematol Oncol