Epstein-Barr virus-associated T/natural killer-cell lymphoproliferative disorders.
Primary infection with Epstein-Barr virus (EBV) is usually asymptomatic and, in a normal host, EBV remains latent in B cells after primary infection for the remainder of life. Uncommonly, EBV can infect T or natural killer (NK) cells in a person with a defect in innate immunity, and EBV infection can cause unique systemic lymphoproliferative diseases (LPD) of childhood. Primary infection in young children can be complicated by hemophagocytic lymphohistiocytosis or fulminant systemic T-cell LPD of childhood. Uncommonly, patients can develop chronic active EBV (CAEBV) disease-type T/NK LPD, which includes CAEBV infection of the systemic form, hydroa vacciniforme-like T-cell LPD, and mosquito-bite hypersensitivity. The clinical course of CAEBV disease-type T/NK LPD can be smoldering, persistent or progressive, depending on the balance between viral factors and host immunity. Aggressive NK-cell leukemia, hydroa vacciniforme-like T-cell lymphoma, or uncommonly extranodal NK/T-cell lymphoma can develop in children and young adults with CAEBV disease-type T/NK-cell LPD. Extranodal T/NK-cell lymphoma is a disease of adults, and its incidence begins to increase in the third decade and comprises the major subtype of T/NK LPD throughout life. Aggressive NK-cell leukemia and nodal T/NK-cell lymphoma of the elderly are fulminant diseases, and immune senescence may be an important pathogenetic factor. This review describes the current progress in identifying different types of EBV-associated T/NK-cell LPD and includes a brief presentation of data from Korea.
Department of Pathology, Ewha Womans University School of Medicine, Seoul, Korea.
Sanghui
S
Park
Young H
YH
Ko
D020031:Epstein-Barr Virus Infections / Q000150:complications* / Q000453:epidemiology; D006801:Humans; D015994:Incidence; D054391:Lymphoma, Extranodal NK-T-Cell / Q000821:virology; D008232:Lymphoproliferative Disorders / Q000453:epidemiology / Q000821:virology*; D009626:Terminology as Topic
Epstein-Barr virus; Korea; T/natural killer cell; lymphoma; lymphoproliferative disease