Table 4.

Changes in the EBV-associated T and NK cell LPD

EBV-associated T and NK cell LPD WHO 2017	EBV-associated T and NK cell LPD 2022 international consensus classification
EBV+ T and NK cell LPD in childhood	EBV+ T and NK cell LPD in childhood
• Chronic active EBV infection -Cutaneous form
Hydroa vacciniforme-like LPD	• Hydroa vacciniforme LPD ▪ Classic form: indolent, self-limited, more common in whites ▪ Systemic form: mild to severe disease, systemic symptoms (fever, lymphadenopathy, liver involvement), more common in Asia and Latin America. Treatment similar to CAEBV disease
Severe mosquito bite allergy	• Severe mosquito bite allergy
-Chronic active EBV infection, systemic form	• Chronic active EBV disease ▪ Systemic disease ▪ Only of T and NK cell type ▪ B cell type is excluded
• Systemic EBV+ T cell lymphoma of childhood	• Systemic EBV+ T cell lymphoma of childhood
Extranodal NK/T cell lymphoma, nasal type	Extranodal NK/T cell lymphoma, nasal type ▪ New genetic findings ▪ Intravascular EBV+ NK cell lymphoma might be a related disease
Aggressive NK cell leukemia	Aggressive NK cell leukemia ▪ Rare cases of EBV-negative are recognized, most common in non-Asians
Primary EBV+ nodal T and NK cell lymphoma, variant of PTCL, NOS	Primary EBV+ nodal T/NK cell lymphoma ▪ More common in elderly and/or immunodeficient patients ▪ Lack nasal involvement ▪ Characteristic genetic findings

LPD, lymphoproliferative disorder. EBV, Epstein–Barr virus; NK, natural killer

PTCL, NOS, peripheral T cell lymphoma, not otherwise specified. Italics means provisional entity