TY - JOUR
T1 - Non-anaplastic peripheral T cell lymphoma in children and adolescents—an international review of 143 cases
AU - Mellgren, K.
AU - Attarbaschi, A.
AU - Abla, O.
AU - Alexander, S.
AU - Bomken, S.
AU - Bubanska, E.
AU - Chiang, A.
AU - Csóka, M.
AU - Fedorova, A.
AU - Kabickova, E.
AU - Kapuscinska-Kemblowska, L.
AU - Kobayashi, R.
AU - Krenova, Z.
AU - Meyer-Wentrup, F.
AU - Miakova, N.
AU - Pillon, M.
AU - Plat, G.
AU - Uyttebroeck, A.
AU - Williams, D.
AU - Wróbel, G.
AU - Kontny, U.
N1 - Publisher Copyright:
© 2016, Springer-Verlag Berlin Heidelberg.
PY - 2016/8/1
Y1 - 2016/8/1
N2 - 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.
AB - 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.
KW - Non-Hodgkin’s lymphoma
KW - Peripheral T cell lymphoma
KW - Prognosis
KW - Subtypes
UR - http://www.scopus.com/inward/record.url?scp=84976260191&partnerID=8YFLogxK
U2 - 10.1007/s00277-016-2722-y
DO - 10.1007/s00277-016-2722-y
M3 - Article
C2 - 27270301
AN - SCOPUS:84976260191
SN - 1432-0584
VL - 95
SP - 1295
EP - 1305
JO - Annals of hematology
JF - Annals of hematology
IS - 8
ER -