TY - JOUR
T1 - Patient characteristics and outcomes in adolescents and young adults with classical Philadelphia chromosome-negative myeloproliferative neoplasms
AU - Boddu, Prajwal
AU - Masarova, Lucia
AU - Verstovsek, Srdan
AU - Strati, Paolo
AU - Kantarjian, Hagop
AU - Cortes, Jorge
AU - Estrov, Zeev
AU - Pierce, Sherry
AU - Pemmaraju, Naveen
N1 - Funding Information:
Funding information The study was supported in part by the MD Anderson Cancer Center Support Grant P30 CA016672 and Award Number P01 CA049639.
Publisher Copyright:
© 2017, Springer-Verlag GmbH Germany, part of Springer Nature.
PY - 2018/1/1
Y1 - 2018/1/1
N2 - Little is known about the outcomes of Philadelphia-negative myeloproliferative neoplasms (MPNs) in adolescents and young adults (AYA). We reviewed all patients with essential thrombocythemia (ET), polycythemia vera (PV), and myelofibrosis (MF) treated at our institution from 1988 to 2016 who were aged 16 to 39 years (AYA) and described their outcomes in comparison to older MPN population. Of 2206 patients, 185 (8.3%) were identified as AYA: 105 (57%) ET, 43 (23%) PV, and 37 (20%) MF. The median age was 33 years [range, 16–39], and median follow-up time 3 years [range, 0.04–25]. JAK2 allele burdens were significantly lower among AYA JAK2V617F-mutated patients in both PV (p = 0.001) and MF (p = 0.005). Seven percent of MPN AYA patients were diagnosed with a thrombotic event at, or prior to, diagnosis. Over the short median follow-up, 4 thrombotic (PV = 1, MF = 3) and 3 leukemia (ET = 2, MF = 1) events occurred. In multivariate analysis, AYA did not predict for thrombotic or transformational events across three cohorts. In the MF cohort, there was a reduced frequency of negative prognostic variables of anemia (p = 0.011) and leukocytosis (p = 0.048) in AYA when compared with non-AYA. Overall survival was significantly superior in the AYA cohorts in all three MPN groups, namely MF (p < 0.001), PV (p < 0.001), and ET (p = 0.002). Our findings suggest that MPN AYA patients exhibit an indolent clinical phenotype characterized by favorable survival outcomes.
AB - Little is known about the outcomes of Philadelphia-negative myeloproliferative neoplasms (MPNs) in adolescents and young adults (AYA). We reviewed all patients with essential thrombocythemia (ET), polycythemia vera (PV), and myelofibrosis (MF) treated at our institution from 1988 to 2016 who were aged 16 to 39 years (AYA) and described their outcomes in comparison to older MPN population. Of 2206 patients, 185 (8.3%) were identified as AYA: 105 (57%) ET, 43 (23%) PV, and 37 (20%) MF. The median age was 33 years [range, 16–39], and median follow-up time 3 years [range, 0.04–25]. JAK2 allele burdens were significantly lower among AYA JAK2V617F-mutated patients in both PV (p = 0.001) and MF (p = 0.005). Seven percent of MPN AYA patients were diagnosed with a thrombotic event at, or prior to, diagnosis. Over the short median follow-up, 4 thrombotic (PV = 1, MF = 3) and 3 leukemia (ET = 2, MF = 1) events occurred. In multivariate analysis, AYA did not predict for thrombotic or transformational events across three cohorts. In the MF cohort, there was a reduced frequency of negative prognostic variables of anemia (p = 0.011) and leukocytosis (p = 0.048) in AYA when compared with non-AYA. Overall survival was significantly superior in the AYA cohorts in all three MPN groups, namely MF (p < 0.001), PV (p < 0.001), and ET (p = 0.002). Our findings suggest that MPN AYA patients exhibit an indolent clinical phenotype characterized by favorable survival outcomes.
KW - Adolescent
KW - Essential thrombocythemia
KW - Myelofibrosis
KW - Myeloproliferative neoplasm
KW - Polycythemia vera
KW - Young adults
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U2 - 10.1007/s00277-017-3165-9
DO - 10.1007/s00277-017-3165-9
M3 - Article
C2 - 29143068
AN - SCOPUS:85034066664
SN - 0939-5555
VL - 97
SP - 109
EP - 121
JO - Annals of Hematology
JF - Annals of Hematology
IS - 1
ER -