title: Clinical features and treatment outcomes of peripheral T-cell lymphoma in children. A current data report from Polish Pediatric Leukemia/Lymphoma Study Group (PPLLSG).
abstract: OBJECTIVE
  Peripheral T-cell lymphomas (PTCL) are lymphoproliferative disorders derived from post-thymic cells, that occur extremely rarely in children. The optimal treatment of pediatric PTCL remains still unclear.
  
  
  METHODS
  Ten children with PTCL from 3 up to 18 years of age registered by the Polish Pediatric Leukemia/Lymphoma Study Group (PPLLSG) were retrospectively analyzed. All patients were treated with different regimens including protocols: for lymphoblastic lymphoma in 7 cases, for anaplastic large cell lymphoma in 1, CHOP in 1. Five of the 10 patients with PTCL were classified as stage II; 4 as stage III and 1 as stage IV due to extralymphatic organs (bone marrow) involvement. Four histological subtypes of PTCL were recognized: extranodal NK/T-cell lymphoma, nasal type (ENTNT), peripheral T-cell lymphoma not otherwise specified (PTCL-NOS), subcutaneous panniculitis-like T-cell lymphoma (SPL), Sezary syndrome (SS). After first-line therapy 9 patients initially achieved complete remission, 4 relapsed, 5 died. One patient achieved remission spontaneously. Three children (1 with stage IV and 2 in relapse) underwent high-dose chemotherapy with allogeneic bone marrow stem cell transplantation and all of them are alive and in CR.
  
  
  RESULTS
  The cumulative probability of 5-year overall survival (OS) for our whole group was 63.9% (95%CI: 35.2-88.2%) with a median follow-up time of 48.4 months (range 24-90+ months). The 5-year event free survival (EFS) was 81%. PTCLs are a heterogeneous and rare group of childhood NHLs.
  
  
  CONCLUSIONS
  According to our experience the standard chemotherapy for precursor lymphomas seems to be a beneficial treatment option for children with PTCL. Allogeneic stem cell transplantation may improve the outcome in selected patients.
authors.affiliation: Department of Pediatrics, Hematology and Oncology, Medical University of Gdansk, Gdansk, Poland. Electronic address: lucynamaciejka@gumed.edu.pl.
authors.forename: Lucyna
authors.initials: L
authors.lastname: Maciejka-Kemblowska
authors.affiliation: Department and Clinic of Pediatric Oncology, Hematology and Bone Marrow Transplantation, Wroclaw Medical University, Wroclaw, Poland.
authors.forename: Radoslaw
authors.initials: R
authors.lastname: Chaber
authors.affiliation: Department and Clinic of Pediatric Oncology, Hematology and Bone Marrow Transplantation, Wroclaw Medical University, Wroclaw, Poland.
authors.forename: Grazyna
authors.initials: G
authors.lastname: Wrobel
authors.affiliation: Department of Developmental Pathology, Medical University of Warsaw, Warszawa, Poland.
authors.forename: Jadwiga
authors.initials: J
authors.lastname: Maldyk
authors.affiliation: Department of Pediatrics, Hematology and Oncology, Medical University of Gdansk, Gdansk, Poland.
authors.forename: Marta
authors.initials: M
authors.lastname: Kozlowska
authors.affiliation: Department and Clinic of Pediatric Oncology, Hematology and Bone Marrow Transplantation, Wroclaw Medical University, Wroclaw, Poland.
authors.forename: Dominika
authors.initials: D
authors.lastname: Kulej
authors.affiliation: Department and Clinic of Pediatric Oncology, Hematology and Bone Marrow Transplantation, Wroclaw Medical University, Wroclaw, Poland.
authors.forename: Bernarda
authors.initials: B
authors.lastname: Kazanowska
authors.affiliation: Department of Pediatric Hematology and Oncology in Zabrze, Medical University of Silesia in Katowice, Poland.
authors.forename: Halina
authors.initials: H
authors.lastname: Bubala
authors.affiliation: Department of Pediatric Oncology, The Children's Memorial Health Institute, Warsaw, Poland.
authors.forename: Bozena
authors.initials: B
authors.lastname: Dembowska-Baginska
authors.affiliation: Department of Pediatric Hematology and Oncology, Wladyslaw Buszkowski Children's Hospital of Kielce, Kielce, Poland.
authors.forename: Grazyna
authors.initials: G
authors.lastname: Karolczyk
authors.affiliation: Department of Pediatric Hematology and Oncology, Nicolaus Copernicus University, Collegium Medicum Bydgoszcz, Bydgoszcz, Poland.
authors.forename: Andrzej
authors.initials: A
authors.lastname: Koltan
authors.affiliation: Department of Pediatric Oncology/Hematology, Institute of Pediatrics, Medical College Jagiellonian University, Krakow, Poland.
authors.forename: Elzbieta
authors.initials: E
authors.lastname: Wyrobek
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007938:Leukemia / Q000473:pathology*; D016399:Lymphoma, T-Cell / Q000188:drug therapy*; D008297:Male; D011044:Poland; D016896:Treatment Outcome
keywords: Children; Non-Hodgkin lymphoma; Peripheral T-cell lymphoma; Treatment