TY - JOUR
T1 - Myeloproliferative neoplasms in the adolescent and young adult population
T2 - A comprehensive review of the literature
AU - Goulart, Hannah
AU - Masarova, Lucia
AU - Mesa, Ruben
AU - Harrison, Claire
AU - Kiladjian, Jean Jacques
AU - Pemmaraju, Naveen
N1 - Publisher Copyright:
© 2024 British Society for Haematology and John Wiley & Sons Ltd.
PY - 2024/7
Y1 - 2024/7
N2 - Myeloproliferative neoplasms (MPN) are characterized by a clonal proliferation of myeloid lineage cells within the bone marrow. The classical BCR-ABL negative MPNs are comprised of polycythaemia vera, essential thrombocythaemia and primary myelofibrosis. Historically, the majority of MPNs are diagnosed in adults older than 60 years of age; however, in recent years, there has been recognition of MPNs in the adolescent and young adult (AYA) population. AYAs with MPN, typically defined as between the ages of 15 and 39 years old, may comprise up to 20% of patients diagnosed with MPN. They demonstrate unique patterns of driver mutations and thrombotic events and remain at risk for progression to more aggressive disease states. Given the likely long length of time they will live with their disease, there is a significant unmet need in identifying well-tolerated and effective treatment options for these patients, particularly with the advent of disease modification. In this review, we provide a comprehensive overview of the clinical features, disease course and management of AYA patients with MPN and, in doing so, highlight key characteristics that distinguish them from their older counterparts.
AB - Myeloproliferative neoplasms (MPN) are characterized by a clonal proliferation of myeloid lineage cells within the bone marrow. The classical BCR-ABL negative MPNs are comprised of polycythaemia vera, essential thrombocythaemia and primary myelofibrosis. Historically, the majority of MPNs are diagnosed in adults older than 60 years of age; however, in recent years, there has been recognition of MPNs in the adolescent and young adult (AYA) population. AYAs with MPN, typically defined as between the ages of 15 and 39 years old, may comprise up to 20% of patients diagnosed with MPN. They demonstrate unique patterns of driver mutations and thrombotic events and remain at risk for progression to more aggressive disease states. Given the likely long length of time they will live with their disease, there is a significant unmet need in identifying well-tolerated and effective treatment options for these patients, particularly with the advent of disease modification. In this review, we provide a comprehensive overview of the clinical features, disease course and management of AYA patients with MPN and, in doing so, highlight key characteristics that distinguish them from their older counterparts.
KW - essential thrombocythaemia
KW - myelofibrosis
KW - myeloproliferative disease
KW - myeloproliferative disorder
KW - polycythaemia vera
UR - http://www.scopus.com/inward/record.url?scp=85195390707&partnerID=8YFLogxK
U2 - 10.1111/bjh.19557
DO - 10.1111/bjh.19557
M3 - Review article
AN - SCOPUS:85195390707
SN - 0007-1048
VL - 205
SP - 48
EP - 60
JO - British Journal of Haematology
JF - British Journal of Haematology
IS - 1
ER -