Clinical features and treatment outcomes of peripheral T-cell lymphoma in children. A current data report from Polish Pediatric Leukemia/Lymphoma Study Group (PPLLSG)

Lucyna Maciejka-Kemblowska; Radoslaw Chaber; Grazyna Wrobel; Jadwiga Maldyk; Marta Kozlowska; Dominika Kulej; Bernarda Kazanowska; Halina Bubala; Bozena Dembowska-Baginska; Grazyna Karolczyk; Andrzej Koltan; Elzbieta Wyrobek

doi:10.1016/j.advms.2016.03.002

Clinical features and treatment outcomes of peripheral T-cell lymphoma in children. A current data report from Polish Pediatric Leukemia/Lymphoma Study Group (PPLLSG)

Adv Med Sci. 2016 Sep;61(2):311-316. doi: 10.1016/j.advms.2016.03.002. Epub 2016 Mar 21.

Affiliations

¹ Department of Pediatrics, Hematology and Oncology, Medical University of Gdansk, Gdansk, Poland. Electronic address: lucynamaciejka@gumed.edu.pl.
² Department and Clinic of Pediatric Oncology, Hematology and Bone Marrow Transplantation, Wroclaw Medical University, Wroclaw, Poland.
³ Department of Developmental Pathology, Medical University of Warsaw, Warszawa, Poland.
⁴ Department of Pediatrics, Hematology and Oncology, Medical University of Gdansk, Gdansk, Poland.
⁵ Department of Pediatric Hematology and Oncology in Zabrze, Medical University of Silesia in Katowice, Poland.
⁶ Department of Pediatric Oncology, The Children's Memorial Health Institute, Warsaw, Poland.
⁷ Department of Pediatric Hematology and Oncology, Wladyslaw Buszkowski Children's Hospital of Kielce, Kielce, Poland.
⁸ Department of Pediatric Hematology and Oncology, Nicolaus Copernicus University, Collegium Medicum Bydgoszcz, Bydgoszcz, Poland.
⁹ Department of Pediatric Oncology/Hematology, Institute of Pediatrics, Medical College Jagiellonian University, Krakow, Poland.

PMID: 27254421
DOI: 10.1016/j.advms.2016.03.002

Abstract

Purpose: 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.

Patients and 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.

Keywords: Children; Non-Hodgkin lymphoma; Peripheral T-cell lymphoma; Treatment.

MeSH terms

Adolescent
Child
Child, Preschool
Female
Humans
Leukemia / pathology*
Lymphoma, T-Cell / drug therapy*
Male
Poland
Treatment Outcome