Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Editorial
. 2024 Sep;103(9):3287-3291.
doi: 10.1007/s00277-024-05920-8. Epub 2024 Aug 7.

Myeloproliferative neoplasms: young patients, current data and future considerations

Marta Sobas  1 , Jean-Christophe Ianotto  2 , Jean-Jacques Kiladjian  3 , Claire Harrison  4
Affiliations
Editorial

Myeloproliferative neoplasms: young patients, current data and future considerations

Marta Sobas et al. Ann Hematol. 2024 Sep.

Abstract

The Philadelphia-negative chronic myeloproliferative neoplasms (MPNs) are clonal hematopoietic stem cell disorders predominantly occurring in elderly, whereas in children and young adults are quite infrequent. Therefore, less is known about clinical presentation, genetic abnormalities, prognosis and best management strategies for this groups of patients. Currently, more cases of younger MPN patients are diagnosed. Nevertheless, diagnosis of MPNs, especially in childhood, may be difficult due to lower incidence of JAK2V617F and CALR mutations and differences in peripheral blood counts between adults and children. Challenges for younger MPN patients are longer life expectances, specific psychosocial need, fertility and pregnancy need and a long term therapy side effect (including second cancers). The most severe MPNs complication is transformation to secondary myelofibrosis (MF) or acute myeloid leukemia (AML). Optimal management of young MPNs remains a challenge as the classical risk scores fail in young MPNs. Moreover, the main objective of young MPNs therapy should be the disease outcome modification. Therefore, international collaborative work between pediatricians and "adult hematologists" is required to measure outcomes and generate protocol of management of young MPNs.

Keywords: Disease modification; Management; Myeloproliferative neoplasms; Pregnancy; Risc scores; Young.

PubMed Disclaimer

References

    1. Arber DA, Orazi A, Hasserjian RP et al (2022) International Consensus classification of myeloid neoplasms and Acute Leukemias: integrating morphologic, clinical, and genomic data. Blood 140(11):1200–1228 - DOI - PubMed - PMC
    1. Boddu P, Masarova L, Verstovsek S et al (2018) Patient characteristics and outcomes in adolescents and young adults with classical Philadelphia chromosome-negative myeloproliferative neoplasms. Ann Hematol 97(1):109–121 - DOI - PubMed
    1. Szuber N, Vallapureddy RR, Penna D et al (2018) Myeloproliferative neoplasms in the young: Mayo Clinic experience with 361 patients age 40 years or younger. Am J Hematol 93(12):1474–1484 - DOI - PubMed
    1. Barzilai M, Kirgner I, Avivi I et al (2019) Characteristics and outcomes of young adults with Philadelphia-negative myeloproliferative neoplasms. Eur J Haematol 102(6):504–508 - DOI - PubMed
    1. Horvat NP, Abdallah EF, Xie Z et al (2024) Young patients with myelofibrosis have distinct clinicomolecular features, favorable prognosis, and commonly exhibit inflammatory comorbidities. Ann Hematol 103(1):117–123 - DOI - PubMed

Publication types

LinkOut - more resources