title: Mature T- and NK-cell non-Hodgkin lymphoma in children and young adolescents.
abstract: Mature T/Natural killer (NK)-cell neoplasms of children and the young adolescent population exhibit higher prevalence in Central and South American and Asian populations and many are associated with Epstein-Barr virus (EBV). They are represented in large part by extranodal T/NK cell lymphomas- nasal-type or extra nasal-type, chronic lymphoproliferative disorders of T/NK cells or chronic active EBV disease, systemic EBV-positive lymphoproliferative disorders of childhood, hydroa vacciniforme-like lymphoma, hepatosplenic T-cell lymphoma and primary cutaneous gamma/delta T-cell lymphoma among others. Many T/NK cell neoplasms in this age group are derived from cells of the innate immune system, in contrast to adults where they are predominantly from the adaptive immune system. The genetic basis of T/NK cell lymphomas in children and young adolescents remains largely unknown. Anthracycline-based regimens and haematopoietic stem cell transplants (allogeneic and autologous) are current treatment modalities, however it is anticipated that novel targeted therapeutic agents will be available in the near future.
authors.affiliation: Department of Pathology and Laboratory Medicine, Hospital of the University of Pennsylvania, University of Pennsylvania, Philadelphia, PA, USA.|Department of Pathology and Laboratory Medicine, The Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: Vinodh
authors.initials: V
authors.lastname: Pillai
authors.affiliation: Section of Hematology-Oncology, Department of Medicine, University of Chicago, Chicago, IL, USA.
authors.forename: Michael
authors.initials: M
authors.lastname: Tallarico
authors.affiliation: Section of Hematology-Oncology, Department of Medicine, University of Chicago, Chicago, IL, USA.
authors.forename: Michael R
authors.initials: MR
authors.lastname: Bishop
authors.affiliation: Department of Pathology and Laboratory Medicine, Hospital of the University of Pennsylvania, University of Pennsylvania, Philadelphia, PA, USA.|Department of Pathology and Laboratory Medicine, The Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: Megan S
authors.initials: MS
authors.lastname: Lim
mesh_terms: D000293:Adolescent; D018943:Anthracyclines / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation / Q000379:methods; D006801:Humans; D007113:Immunity, Innate; D016130:Immunophenotyping; D054391:Lymphoma, Extranodal NK-T-Cell / Q000175:diagnosis / Q000628:therapy*; D016399:Lymphoma, T-Cell / Q000175:diagnosis / Q000628:therapy*; D008297:Male; D058990:Molecular Targeted Therapy / Q000639:trends
chemical_list: D018943:Anthracyclines
keywords: paediatric mature T-cell NK-cell lymphoma