There is no single diagnostic marker for polycythaemia vera (PV). The
Polycythemia Vera Study Group established diagnostic criteria more tha
n twenty years ago. Since some new laboratory and clinical investigati
ons have developed, these criteria can now be updated. It is proposed
that the overall pattern of major and minor criteria should remain. A
raised red cell mass (greater than 25% above the patient's mean normal
predicted value based on surface area) and absence of a cause of seco
ndary polycythaemia are essential criteria. Then either palpable splen
omegaly or the presence of a marker of clonal haemopoiesis would suppo
rt the diagnosis of PV. In the absence of both of these latter major c
riteria, at least two minor criteria must be present to secure the dia
gnosis. These are: platelet count >400 x 10(9)/l; neutrophil count >10
x 10(9)/l; splenomegaly demonstrated by a scanning technique; charact
eristic BFU-E growth or reduced serum erythropoietin value.