ABSTRACT
This chapter describes the pathogenesis, contemporary diagnosis, risk stratification and treatment of the myeloproliferative neoplasms (MPNs) and related neoplasm. No specific epidemiological risk factors have been associated with any of the classic MPNs, though there are multiple well-characterized pedigrees with autosomal dominant pattern of susceptibility of multiple MPNs in the same family. All patients with MPN have a significant risk of thrombotic events, which is the highest for polycythaemia vera. Several validated risk stratification models for MPN have been developed, which allows for an assessment of the risk of survival, from risk of thrombosis and from risk of transformation. The drug's major effect appears to be suppression of the inflammatory cytokine production, a major feature resulting in MPN-related symptoms, plus myelosuppression. Chronic neutrophilic leukaemia is a rare MPN subtype which shares overlapping clinical features with myelodysplastic syndromes/MPN subtype, Atypical chronic myeloid leukaemia (aCML).
