Ph-Negative Myeloproliferative Neoplasms: Diagnosis and Treatment Challenges in Russia (the Case of Saint Petersburg)

Abstract

Ph-negative myeloproliferative neoplasms (MPN) are rare oncohematological diseases characterized by long duration and indolence. World epidemiological data on these diseases considerably vary depending on geographical area and time frame of the study. The breakthrough in the understanding of MPN pathogenesis, observed in the early 2000s, enabled to elaborate approaches to differential diagnosis and treatment of Ph-negative MPNs as well as to improve their prognosis. Although these approaches are specified in the Russian clinical guidelines, physicians still face challenges in their implementation in practice. The present review provides a detailed description and analysis of literature data on epidemiology, pathogenesis, and principles of Ph-negative MPN diagnosis and treatment. It also describes the situation in Saint Petersburg as an example of existing challenges in management of patients with Ph-negative MPNs in Russia and offers potential solutions.