ABSTRACT
Polycythemia vera (PV) is characterized by clonal proliferation of bone marrow progenitors leading to abnormal production of erythroid cell line that is independent of physiological growth factor erythropoietin (EPO). This notion led investigators to examine downstream receptors events and pathogenesis. The diagnosis of this disease has advanced considerably with the recent discoveryofacquiredmutationsofJanuskinase2 (JAK2 )geneinthevastmajorityofpatients.1-6
Early studies in untreated patients found a high incidence of thrombotic events and a life expectancy of about 18 months after diagnosis. 7 Cytoreductive treatments of blood hyperviscosity by phlebotomy or chemotherapy as well as antithrombotic therapy have been shown to dramatically reduce the number of vascular events, though hematological transformations to post-polycythemic myelofibrosis (MF) and acute leukemia still represent a major cause of death. 8
Inthischapter,weundertakeashortreview of the seminal studies contributing to the status quo up to the year 2000; challenges and unanswered questions at the beginning of this century; and discuss the most recent developments in the current state-of-the-art and some speculations for the future.
