Polycythaemia may complicate or be the presenting feature of a wide va
riety of different pathologies. Early diagnosis and treatment of prima
ry polycythaemia will significantly reduce the morbidity and mortality
associated with this condition. Patients with a raised packed cell vo
lume are divided into those with a raised red cell. mass (absolute pol
ycythaemia), and those with a red cell mass within their normal range
(apparent polycythaemia). A standard investigative approach of an abso
lute polycythaemia enables patients with primary and secondary polycyt
haemia to be identified, leaving a group termed idiopathic erythrocyto
sis. There are a number of physiological situations and pathological e
vents associated with idiopathic erythrocytosis and apparent polycytha
emia. Careful follow-up of both groups of these patients is essential
to identify possible causative mechanisms.