Les néoplasies myéloprolifératives en 2022
Une revue concise
Résumé
Les néoplasies myéloprolifératives (NMP) sans chromosome Philadelphie (Ph-) sont constituées d’un groupe hétérogène d’affections des cellules souches hématopoïétiques myéloïdes qui comprennent la polycythémie vraie (PV), la thrombocytémie essentielle (TE) et la myélofibrose primitive (MF). Les NMP sont caractérisées par des symptômes constitutionnels et d’autres symptômes liés à la maladie, un risque accru d’événements thrombotiques et hémorragiques et une propension à se transformer en leucémie myéloïde aiguë (LMA). Les progrès faits dans notre compréhension de la physiopathologie moléculaire des NMP ont conduit à l’amélioration des outils pronostiques et à une stratification des risques de plus en plus personnalisée. Dans la PV, l’interféron (IFN) a connu un regain d’intérêt en raison de son potentiel à cibler directement le clone malin et à exercer un effet modificateur sur la maladie. Dans la MF, l’introduction des inhibiteurs des janus kinases (JAK) a considérablement modifié le paysage thérapeutique au cours de la dernière décennie. Le développement continuel dans le domaine de la thérapie par inhibiteurs des JAK, ainsi que l’étude de nouvelles voies de signalisation, sont prometteurs pour améliorer les réponses hématologiques, réduire le fardeau global de la maladie, améliorer la qualité de vie et élargir l’application à une cohorte plus large de patients.
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