Hipertensão portal não cirrótica em pacientes com policitemia vera

Abstract

This literature reviewaddresses the epidemiological, pathogenetic, clinical, diagnostic, and therapeutic aspects of Polycythemia Vera (PV) and its association with splanchnic vein thrombosis (SVT), which is an important cause of non-cirrhotic portal hypertension in adults. Myeloproliferative neoplasms (MPNs), including Polycythemia Vera (PV), Essential Thrombocythemia (ET), and Primary Myelofibrosis (PMF), are hematologic disorders characterized by the clonal proliferation of myeloid cells and are frequently associated with thrombotic complications. SVT may be the initial manifestation of MPNs in a significant proportion of patients, with emphasis on the presence of the JAK2 V617F mutation and the association of factors such as age and hypercoagulable disorders being relevant in the pathogenesis of SVT. Diagnosis involves investigating genetic mutations and excluding other causes, such as liver diseases. Treatment includes systemic anticoagulation, with preference for medications like warfarin or DOACs, depending on the cost and the patient’s preference, as there is still no consensus in the literature regarding the superiority of one medication over another. Aspirin therapy and phlebotomy have been shown to improve thrombotic-free survival in patients with PV. However, combined therapies with anticoagulants and antiplatelet agents should be avoided due to the risk of bleeding. The treatment of patients with MPNs focuses on preventing thrombotic complications using cytoreductive agents, with allogeneic bone marrow transplantation being a more reserved definitive treatment option for cases with unfavorable prognosis.