Mechanisms of cerebral venous sinus thrombosis due to essential thrombocythemia: Current status and future perspectives.

Essential Thrombocythemia (ET) is a myeloproliferative neoplasm driven by JAK2V617F, CALR, and MPL mutations, characterized by persistent thrombocytosis and hypercoagulability. Cerebral Venous Sinus Thrombosis (CVST) represents a severe complication of ET, yet the underlying mechanisms remain unclear. Genetically, the JAK2V617F mutation activates the JAK-STAT pathway, inducing platelet hyperactivation, neutrophil extracellular trap formation, and the release of platelet-derived microparticles, which alter blood rheology and promote thrombosis. Mutations in CALR and MPL accelerate megakaryopoiesis, while erythrocyte dysfunction exacerbates hyperviscosity. Anatomical variations in the cerebral venous sinuses synergize with these abnormalities to create a prothrombotic microenvironment. Diagnostically, MRI/MRV is the first choice, with the primary challenge being the differentiation of venous sinus hypoplasia from thrombosis in ET patients, combined with the detection of the JAK2 mutation for confirmation. This review summarizes the genetic and cellular mechanisms linking ET to CVST, discusses tailored diagnostic strategies and therapies (JAK inhibitors, anticoagulants), and highlights the need for further research to clarify the interplay between molecular abnormalities and vascular factors, aiming to optimize clinical management.