Leukocytosis and MPNs: Where do we stand?

The Philadelphia-negative myeloproliferative neoplasms (MPNs)-essential thrombocythemia (ET), polycythemia vera (PV) and myelofibrosis (MF), are characterized by a propensity for thrombotic events and variable risks for transformation to MF (for ET and PV) and acute leukemia. Leukocytosis, which serves a minor criterion for the diagnosis of MF, is present in a significant portion of patients with MPNs. The relation and impact of leukocytosis on disease course and outcomes of patients with MPNs has been studied in multiple, large retrospective and prospective studies. Despite this, the association of leukocytosis and thrombosis, fibrosis and leukemic transformation remains unclear. This article details the published investigations regarding the impact of leukocytosis in MPNs and explores the changing role of leukocytosis in disease prognostication as increasing emphasis is placed on molecular and genetic studies.