02005nam a2200253 4500 ESSALUD 20260821144938.0 ta t pe ||||| |||| 00| 0 spa d BMG eng Plo, Isabelle Autor 54275 Myeloproliferative neoplasms páginas: 788-802 Classic myeloproliferative neoplasms, including essential thrombocythemia, polycythemia vera, and primary myelofibrosis, are chronic, clonal hematopoietic stem-cell disorders. These disorders are driven by gain-of-function mutations in the genes Janus kinase 2 (JAK2), calreticulin (CALR), or the thrombopoietin receptor (MPL) that activate cytokine signaling. These mutations arise decades before clinical disease develops and confer a clonal advantage that is further shaped by comutations in epigenetic, splicing, or signaling genes. Inflammation enhances clonal dominance, favoring the development of myelofibrosis and thrombotic complications. Disease evolution may culminate in secondary acute myeloid leukemia, which has a poor prognosis. Current therapies primarily aim to control symptoms, thrombosis, and splenomegaly, but they have limited disease-modifying effects, except for pegylated interferon alfa and JAK2 inhibitors in some patients. Emerging therapies that selectively target mutant CALR and JAK2 V617F using immunotherapy and selective inhibitors could be a breakthrough in the treatment of persons with myeloproliferative neoplasms, with the expectation of achieving durable disease modification and potentially clonal eradication. CÁNCER 1231 TRATAMIENTOS EN ONCOLOGÍA 53734 ENFERMEDAD INFLAMATORIA 53667 LEUCEMIA 33873 LINFOMA 39360 Vainchenker, William 54276 22717 22650 Massachusetts NEJM Group NEJM12 The New England Journal of Medicine ESSALUD 0028-4793 ARTICULOS 2026-08-21 sqb 22856 22856