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Clinical reviews
Myeloproliferative Neoplasms
INSERM Unité Mixte de Recherche 1287, Gustave Roussy
PDF202615 pages6.0 MB
This review article discusses classic myeloproliferative neoplasms (MPNs) such as essential thrombocythemia, polycythemia vera, and primary myelofibrosis. These are clonal hematopoietic stem-cell disorders driven by mutations in JAK2, CALR, or MPL, which activate cytokine signaling. The article details how these mutations arise, the role of comutations and inflammation in disease progression, and the potential for evolution to acute myeloid leukemia. Current therapies are discussed, along with emerging treatments targeting specific mutations and immunotherapy approaches aimed at achieving disease modification and potential clonal eradication.
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