title: Epstein-Barr virus-positive systemic NK/T-cell lymphomas in children: report of six cases.
abstract: OBJECTIVE
  The World Health Organization lymphoma classification recognizes two different Epstein-Barr virus (EBV)-positive T-cell lymphoproliferative disorders of childhood: systemic EBV-positive T-cell lymphoproliferative disease of childhood, and hydroa vacciniforme-like lymphoma, which is more prevalent in Asia and Latin America. The aim of this study was to characterize six cases of paediatric EBV-positive peripheral T-cell lymphoma with distinct features.
  
  
  RESULTS
  All cases were male, with a median patient age of 9 years (range: 5-17 years). Most of them presented suddenly with fever, weight loss, hepatosplenomegaly, peripheral lymphadenopathy, and high lactate dehydrogenase (LDH) levels. Moreover, gut, lung or soft tissues of the abdominal wall were also affected in four cases. Partial to total replacement of the lymph node by pleomorphic infiltration of atypical neoplastic cells was found in all cases. Vasculitis and geographical areas of necrosis were seen in three and four cases, respectively. Neoplastic cells showed expression of EBV-encoded RNA, T-cell markers (CD2 and CD3), and cytotoxic markers (TIA1, granzyme-B, and perforin). CD56 and T-cell receptor -γ were expressed in one case each. TCR-BF1, CD4, CD8 and anaplastic lymphoma kinase were negative. In all cases, the disease progressed rapidly, causing death of the patient, with a median survival of 7.1 months (range: 1-13 months).
  
  
  CONCLUSIONS
  These cases probably represent a solid form of systemic EBV-positive T-cell lymphoproliferative disease of childhood, which requires identification and the development of appropriate therapy.
authors.affiliation: Lymphoma Group, Molecular Pathology Programme, Spanish National Cancer Research Centre (CNIO), Madrid, Spain. smrodriguez@cnio.es
authors.forename: Socorro M
authors.initials: SM
authors.lastname: Rodríguez-Pinilla
authors.forename: Carlos
authors.initials: C
authors.lastname: Barrionuevo
authors.forename: Juan
authors.initials: J
authors.lastname: García
authors.forename: María
authors.initials: M
authors.lastname: de los Ángeles
authors.forename: Raquel
authors.initials: R
authors.lastname: Pajares
authors.forename: Sandro
authors.initials: S
authors.lastname: Casavilca
authors.forename: Jaime
authors.initials: J
authors.lastname: Montes
authors.forename: Antonio
authors.initials: A
authors.lastname: Martínez
authors.forename: Santiago
authors.initials: S
authors.lastname: Montes-Moreno
authors.forename: Lydia
authors.initials: L
authors.lastname: Sánchez
authors.forename: Miguel Ángel
authors.initials: MÁ
authors.lastname: Piris
mesh_terms: D000293:Adolescent; D014408:Biomarkers, Tumor / Q000032:analysis; D002648:Child; D002675:Child, Preschool; D020031:Epstein-Barr Virus Infections / Q000150:complications*; D006801:Humans; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D007694:Killer Cells, Natural / Q000473:pathology*; D016411:Lymphoma, T-Cell, Peripheral / Q000378:metabolism / Q000473:pathology* / Q000821:virology*; D008297:Male
chemical_list: D014408:Biomarkers, Tumor