Abstract
Epstein-Barr virus-associated T/NK-cell lymphoproliferative disorder (EBV-T/NK LPD) of children and young adults generally overlaps with the nosological term of severe chronic active EBV infection (CAEBV). We propose clinicopathological categorization of EBV-T/NK LPD based on pathological evaluation and molecular data into : 1) Category A1, polymorphic LPD without clonal proliferation of EBV-infected cells ; 2) Category A2, polymorphic LPD with clonality ; 3) Category A3, monomorphic LPD (T-cell or NK cell lymphoma/leukemia) with clonality. However, CAEBV with all A1, A2, and A3 is a high-mortality and high-morbidity disease.[Skin Cancer (Japan) 2009 ; 24 : 212-217]