Annals of Hematology

, Volume 97, Issue 1, pp 109–121 | Cite as

Patient characteristics and outcomes in adolescents and young adults with classical Philadelphia chromosome-negative myeloproliferative neoplasms

  • Prajwal Boddu
  • Lucia Masarova
  • Srdan Verstovsek
  • Paolo Strati
  • Hagop Kantarjian
  • Jorge Cortes
  • Zeev Estrov
  • Sherry Pierce
  • Naveen Pemmaraju
Original Article
First Online:
Received:
Accepted:

Abstract

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.

Keywords

Essential thrombocythemia Polycythemia vera Myelofibrosis Young adults Adolescent Myeloproliferative neoplasm 
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Notes

Author contribution

PB, LM, SV, PS, HK, JC, ZE, SP, and NP were all involved in designing the study, collecting, and writing the manuscript. All authors provided critical analysis, and each has reviewed the manuscript.

Funding information

The study was supported in part by the MD Anderson Cancer Center Support Grant P30 CA016672 and Award Number P01 CA049639.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Supplementary material

277_2017_3165_MOESM1_ESM.docx (14 kb)
ESM 1 (DOCX 13 kb)

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2017

Authors and Affiliations

  • Prajwal Boddu
    • 1
  • Lucia Masarova
    • 1
  • Srdan Verstovsek
    • 1
  • Paolo Strati
    • 1
  • Hagop Kantarjian
    • 1
  • Jorge Cortes
    • 1
  • Zeev Estrov
    • 1
  • Sherry Pierce
    • 1
  • Naveen Pemmaraju
    • 1
  1. 1.Department of LeukemiaUniversity of Texas, M. D. Anderson Cancer CenterHoustonUSA

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