Non-anaplastic peripheral T cell lymphoma in children and adolescents—an international review of 143 cases

K. Mellgren, A. Attarbaschi, O. Abla, S. Alexander, S. Bomken, E. Bubanska, A. Chiang, M. Csóka, A. Fedorova, E. Kabickova, L. Kapuscinska-Kemblowska, R. Kobayashi, Z. Krenova, F. Meyer-Wentrup, N. Miakova, M. Pillon, G. Plat, A. Uyttebroeck, D. Williams, G. WróbelU. Kontny

Onderzoeksoutput: Bijdrage aan tijdschriftArtikelpeer review

46 Citaten (Scopus)


Peripheral T cell lymphomas (PTCL) are rare in children and adolescents, and data about outcome and treatment results are scarce. The present study is a joint, international, retrospective analysis of 143 reported cases of non-anaplastic PTCL in patients <19 years of age, with a focus on treatment and outcome features. One hundred forty-three patients, between 0.3 and 18.7 years old, diagnosed between 2000 and 2015 were included in the study. PTCL not otherwise specified was the largest subgroup, followed by extranodal NK/T cell lymphoma, hepatosplenic T cell lymphoma (HS TCL), and subcutaneous panniculitis-like T cell lymphoma (SP TCL). Probability of overall survival (pOS) at 5 years for the whole group was 0.56 ± 0.05, and probability of event-free survival was (pEFS) 0.45 ± 0.05. Patients with SP TCL had a good outcome with 5-year pOS of 0.78 ± 0.1 while patients with HS TCL were reported with 5-year pOS of only 0.13 ± 0.12. Twenty-five percent of the patients were reported to have a pre-existing condition, and this group had a dismal outcome with 5-year pOS of 0.29 ± 0.09. The distribution of non-anaplastic PTCL subtypes in pediatric and adolescent patients differs from what is reported in adult patients. Overall outcome depends on the subtype with some doing better than others. Pre-existing conditions are frequent and associated with poor outcomes. There is a clear need for subtype-based treatment recommendations for children and adolescents with PTCL.

Originele taal-2Engels
Pagina's (van-tot)1295-1305
Aantal pagina's11
TijdschriftAnnals of hematology
Nummer van het tijdschrift8
StatusGepubliceerd - 1 aug. 2016


Duik in de onderzoeksthema's van 'Non-anaplastic peripheral T cell lymphoma in children and adolescents—an international review of 143 cases'. Samen vormen ze een unieke vingerafdruk.

Citeer dit