title: Peripheral T-cell lymphoma in children and adolescents: a single-institution experience.
abstract: BACKGROUND
  Peripheral T-cell lymphoma (PTCL) is rare in pediatric patients and is associated with worse outcome compared with other pediatric non-Hodgkin lymphomas. We report our institutional experience over a 10-year period.
  
  
  METHODS
  Patients were identified through the institutional oncology database. Data were abstracted through a detailed retrospective review of patient charts. Those with isolated cutaneous T-cell lymphoma were excluded from this analysis.
  
  
  RESULTS
  Thirteen patients were diagnosed with PTCL during the 10-year period. All presented with advanced disease. According to the WHO PTCL classification, 7 patients had PTCL not otherwise specified, 3 had hepatosplenic T-cell lymphoma and 3 had T-cell posttransplant lymphoproliferative disease. Six of the patients had previously received or were receiving immunosuppressive therapy at the time of their diagnosis. Patients were treated with various chemotherapeutic regimens, including B-cell non-Hodgkin lymphomas and T-cell acute lymphoblastic leukemia-like therapy. Patients who had refractory or recurrent disease were changed to alternative therapy. Eight patients underwent stem cell transplantation, 7 allogeneic and 1 autologous. Of the 13 patients, 7 died. Three patients died from disease progression, 3 died from treatment-related mortality, and 1 patient died from cardiac rejection. Six patients (46%) are alive and disease free at a median of 21 months (2 to 79 mo).
  
  
  CONCLUSIONS
  Children with PTCL have an unfavorable outcome. A standard approach to management of pediatric PTCL needs to be established to improve outcome. Because of the rarity of this condition, this will require collaborative studies.
authors.affiliation: Department of Paediatrics, Division of Haematology/Oncology, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.
authors.forename: Rabah
authors.initials: R
authors.lastname: Al Mahmoud
authors.forename: Sheila
authors.initials: S
authors.lastname: Weitzman
authors.forename: Tal
authors.initials: T
authors.lastname: Schechter
authors.forename: Bo
authors.initials: B
authors.lastname: Ngan
authors.forename: Mohammed
authors.initials: M
authors.lastname: Abdelhaleem
authors.forename: Sarah
authors.initials: S
authors.lastname: Alexander
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D016867:Immunocompromised Host; D007166:Immunosuppressive Agents / Q000009:adverse effects; D007223:Infant; D016411:Lymphoma, T-Cell, Peripheral / Q000188:drug therapy / Q000453:epidemiology* / Q000601:surgery; D008297:Male; D009426:Netherlands / Q000453:epidemiology; D011183:Postoperative Complications / Q000453:epidemiology; D011379:Prognosis; D012189:Retrospective Studies; D016019:Survival Analysis
chemical_list: D007166:Immunosuppressive Agents