Janus kinase inhibitors in myeloproliferative neoplasms: Clinical applications

Abstract

Hyperactive Janus kinase 2 signalling is a key molecular event in polycythaemia, essential thrombocythaemia and myelofibrosis. This is associated with the V617F mutation in the Janus kinase 2 gene of many patients with myeloproliferative disease. Ruxolitinib is the first Janus kinase inhibitor to be licensed in Australia for the treatment of myelofibrosis. Ruxolitinib can cause rapid and sustained splenic shrinkage in up to 42% of patients with higher risk myelofibrosis, however it does not change the risk of leukaemic transformation. Treatment with ruxolitinib can be limited by significant anaemia.