title: EBV-associated T/NK-cell lymphoproliferative diseases in nonimmunocompromised hosts: prospective analysis of 108 cases.
abstract: EBV-associated T/NK-cell lymphoproliferative disease (T/NK-LPD) is defined as a systemic illness characterized by clonal proliferation of EBV-infected T or NK cells. We prospectively enrolled 108 nonimmunocompromised patients with this disease (50 men and 58 women; median onset age, 8 years; age range, 1-50 years) evidenced by expansion of EBV(+) T/NK cells in the peripheral blood; these were of the T-cell type in 64 cases and of the NK-cell type in 44, and were clinically categorized into 4 groups: 80 cases of chronic active EBV disease, 15 of EBV-associated hemophagocytic lymphohistiocytosis, 9 of severe mosquito bite allergy, and 4 of hydroa vacciniforme. These clinical profiles were closely linked with the EBV(+) cell immunophenotypes. In a median follow-up period of 46 months, 47 patients (44%) died of severe organ complications. During the follow-up, 13 patients developed overt lymphoma or leukemia characterized by extranodal NK/T-cell lymphoma and aggressive NK-cell leukemia. Fifty-nine received hematopoietic stem cell transplantation, 66% of whom survived. Age at onset of disease (≥ 8 years) and liver dysfunction were risk factors for mortality, whereas patients who received transplantation had a better prognosis. These data depict clinical characteristics of systemic EBV(+) T/NK-LPD and provide insight into the diagnostic and therapeutic approaches for distinct disease.
authors.affiliation: Departments of Virology, Nagoya University Graduate School of Medicine, Japan. hkimura@med.nagoya-u.ac.jp
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Kimura
authors.forename: Yoshinori
authors.initials: Y
authors.lastname: Ito
authors.forename: Shinji
authors.initials: S
authors.lastname: Kawabe
authors.forename: Kensei
authors.initials: K
authors.lastname: Gotoh
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Takahashi
authors.forename: Seiji
authors.initials: S
authors.lastname: Kojima
authors.forename: Tomoki
authors.initials: T
authors.lastname: Naoe
authors.forename: Shinichi
authors.initials: S
authors.lastname: Esaki
authors.forename: Atsushi
authors.initials: A
authors.lastname: Kikuta
authors.forename: Akihisa
authors.initials: A
authors.lastname: Sawada
authors.forename: Keisei
authors.initials: K
authors.lastname: Kawa
authors.forename: Koichi
authors.initials: K
authors.lastname: Ohshima
authors.forename: Shigeo
authors.initials: S
authors.lastname: Nakamura
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D004279:DNA, Viral / Q000235:genetics; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000473:pathology* / Q000821:virology; D005260:Female; D005500:Follow-Up Studies; D004854:Herpesvirus 4, Human / Q000235:genetics / Q000276:immunology / Q000302:isolation & purification*; D006801:Humans; D016867:Immunocompromised Host; D007223:Infant; D007694:Killer Cells, Natural / Q000276:immunology* / Q000473:pathology / Q000821:virology; D008232:Lymphoproliferative Disorders / Q000209:etiology* / Q000401:mortality / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D016133:Polymerase Chain Reaction; D011379:Prognosis; D011446:Prospective Studies; D015996:Survival Rate; D055815:Young Adult
chemical_list: D004279:DNA, Viral