Pathogenesis of polycythaemia vera

Abstract

Polycythaemia vera (PV) is thought to result from clonal expansion of a transformed multipotent stem cell. Progenitors from patients with PV display abnormal responses to several growth factors, suggesting the presence of a defect in a signalling pathway common to different growth factors. A number of approaches are now focused on defining the molecular lesion or lesions. Identification of causal genes will be of considerable interest both to clinicians, who currently lack a specific and sensitive diagnostic test, and to scientists interested in fundamental issues of stem cell behaviour.