POLYCYTHEMIA VERA: PRESENTATION AND MANAGEMENT

Abstract: Background: Polycythemia vera (PV), alongside primary myelofibrosis (MF) and essential thrombocythemia (ET), is a classic Philadelphia chromosome-negative myeloproliferative neoplasm (MPN) characterized primarily by an expanded red platelet mass. Patients with PV have excessive proliferation of erythroid as well as myeloid and megakaryocytic components in the bone marrow, which result in high red blood cell, white blood cell (WBC), and platelet counts. Clinically, patients with PV may encounter manifestations, for example, pruritus, fatigue, night sweats, bone pain, thrombosis, and bleeding Methodology: We conducted this review using a comprehensive search of MEDLINE, PubMed, and EMBASE, January 1985, through February 2017. The following search terms were used: polycythemia Vera, presentation, complication, diagnosis, and management of Polycythemia Vera, JAK2 mutation, targeted therapy Aim: In this review, we aim to study the clinical presentation, complication, diagnosis, and advances in management of polycythemia Vera. Conclusion: The discovery of JAK2 mutations as the underlying molecular basis for PV has enormously expanded our comprehension of the pathogenesis of PV and has allowed for the development of targeted treatments. As of now, studies are evaluating the clinical advantages of JAK2 inhibitors and are demonstrating promising outcomes for the treatment of this debilitating disease. Further studies will mostly focus around which patients with PV will profit most from the utilization of targeted treatments and how these new treatments compare with present treatment standards. The best treatment for every patient will be one that is well endured while improving symptoms and quality of life, and in such manner, targeted treatments will be valuable tools. Keywords: Polycythemia Vera, JAK2 mutation, hematologic patholog