J Korean Med Sci.  2008 Apr;23(2):185-192. 10.3346/jkms.2008.23.2.185.

The Spectrum of Epstein-Barr Virus-Associated Lymphoproliferative Disease in Korea: Incidence of Disease Entities by Age Groups

• Cho EY
• Kim KH
• Kim WS
• Yoo KH
• Koo HH
• Ko YH

• KMID: 1713447
• DOI: http://doi.org/10.3346/jkms.2008.23.2.185

Abstract

This study is to identify the spectrum of Epstein-Barr virus (EBV)-positive lymphoproliferative diseases (LPD) and relationships between these diseases in Korea. The EBV status and clinicopathology of 764 patients, including acute EBV-associated hemophagocytic lymphohistiocytosis (EBV-HLH), chronic active EBV (CAEBV) infections, B-LPD arising in chronic latent EBV infection, T & natural killer (NK) cell non-Hodgkin's lymphomas (NHL), B-NHLs, and Hodgkin's lymphomas (HD), were analyzed. T or NK cell NHLs were the most common forms of EBV-positive NHLs (107/167, 64%); among these, nasal-type NK/T cell lymphomas were the most common (89/107, 83%). According to the age, Burkitt's lymphoma was the most common in early childhood; in teenagers, chronic (active) EBV infection-associated LPD was the most common type. The incidence of NK/T cell lymphoma began to increase from the twenties and formed the major type of EBV-associated tumor throughout life. Diffuse large B cell lymphoma formed the major type in the sixties and seventies. In conclusion, primary infections in early childhood are complicated by the development of CAEBV infections that are main predisposing factors for EBV-associated T or NK cell malignancies in young adults. In old patients, decreased immunity associated with old age and environmental cofactors may provoke the development of peripheral T cell lymphoma, unspecified, and diffuse large B cell lymphoma.

Keyword

Lymphoma; Epstein-Barr Virus; Lymphohistiocytosis, Hemophagocytic

MeSH Terms

Adolescent
Adult
Age Factors
Aged
Aged, 80 and over
Child
Child, Preschool
Epstein-Barr Virus Infections/*diagnosis/*epidemiology
Herpesvirus 4, Human/*metabolism
Humans
Infant
Korea
Lymphoproliferative Disorders/*diagnosis/epidemiology/*virology
Middle Aged
Treatment Outcome

Figure

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  Fig. 1 Cell lineage and histological types among EBV-positive lymphomas.

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  Fig. 2 The change of common histological types of EBV-positive lymphoproliferative disease according to age. CEBV-B, chronic EBV infection associted B-LPD; CAEBV-T/NK, chronic active EBV infection-associated T or NK cell LPD.

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  Fig. 3 Survival curve of patients with EBV-positive lymphoproliferative disease. CEBV-B, chronic EBV infection associated B-LPD; CAEBV-T/NK, chronic active EBV infection associated T or NK cell LPD; TCL, peripheral T-cell lymphoma.

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  Fig. 4 The relation between EBV infection and development of EBV-associated lymphoproliferative disease by age group. HD, Hodgkin's lymphoma; ANKL, aggressive NK cell leukemia; DLBCL, diffuse large B cell lymphoma; PTCL, peripheral T cell lymphoma. *, environmental cofactor.

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