Polycythemia vera, essential thrombocytosis, and primary myelofibrosis are hematopoietic stem-cell disorders characterized
by clonal dominance and unregulated increase in circulating erythrocytes, leukocytes, or platelets alone or in combination.
An activating mutation (V617F) in the gene for JAK2 (Janus kinase 2) may explain the shared clinical features of these 3 disorders.
Polycythemia vera is the potential ultimate clinical phenotype of the JAK2 V617F mutation and, as a corollary, is the most common of the 3 disorders.