Myeloproliferative neoplasms: young patients, current data and future considerations.

Autor: Sobas M; Department of Hematology, Blood Neoplasms and Bone Marrow Transplantation, Wroclaw Medical University, Wroclaw, Poland. marta.sobas@gmail.com., Ianotto JC; Department of Hematology, Institute of Oncology and Hematology, University Hospital, Brest, France., Kiladjian JJ; Assitance Publique - Hôpitaux de Paris, Hôpital Saint-Louis, Université de Paris, Inserm, Paris, France., Harrison C; Department of Hematology, Guy's and St Thomas' National Health Service Foundation Trust, London, UK.
Jazyk: angličtina
Zdroj: Annals of hematology [Ann Hematol] 2024 Sep; Vol. 103 (9), pp. 3287-3291. Date of Electronic Publication: 2024 Aug 07.
DOI: 10.1007/s00277-024-05920-8
Abstrakt: 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.
(© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)
Databáze: MEDLINE
Externí odkaz: