The Pathogenesis, Diagnosis, and Treatment of Polycythaemia Vera

摘要: Polycythaemia vera (PV) was first described in 1892 by Vazquez [1] a case report describing patient with ruddy cyanosis, splenomegaly, and an increased red cell count not associated congenital form of heart disease. A decade later 1903 it defined more clearly Osler [2], the disease then became known as Vasquez–Osler until this eponymous term superseded PV. PV together essential thrombocythaemia (ET) primary myelofibrosis (PMF) (Chap. 12) represent overlapping spectrum clonal haematological disorders called human myeloproliferative neoplasms (MPN). The MPNs were grouped together, along chronic myeloid leukaemia (CML), Dameshek his seminal paper 1951 [3]. CML is now generally considered distinct entity, but shares several features other MPNs. All these result from acquired genetic changes haematopoietic stem compartment are characterized proliferation various cells lineages. They also all share propensity to develop into acute (AML), albeit varying incidence, and, will be next, majority demonstrate abnormalities intracellular signalling. Taking characteristics MPN therefore provide vivo model systems study multistep development AML. In addition, full terminal differentiation lineages, they allow effects oncogenic mutations on normal homeostasis before complicated cooperating which block differentiation.