The pathogenesis, diagnosis, and treatment of polycythaemia vera

Abstract

Polycythaemia vera (PV) was first described in 1892 by Vazquez [1] in a case report describing a patient with ruddy cyanosis, splenomegaly, and an increased red cell count not associated with a congenital form of heart disease. A decade later in 1903 it was defined more clearly by Osler [2], and the disease then became known as Vasquez-Osler disease until this eponymous term was superseded by PV. PV together with essential thrombocythaemia (ET) and primary myelofibrosis (PMF) (Chap. 12) represent an overlapping spectrum of clonal haematological disorders called the human myeloproliferative neoplasms (MPN). The MPNs were first grouped together, along with chronic myeloid leukaemia (CML), by D..