Update on diagnosis and management in essential thrombocythemia
DUAN Ming-hui
Abstract：Essential thrombocythemia(ET) is often regarded as the most indolent of "BCR-ABL1-negative MPN',a disorder which characterized by stem cell-derived clonal myeloproliferation with mutually exclusive JAK2,CALR and MPL mutations.Accurate distinction between ET and pre-fibrotic/early primary myelofibrosis (pre PMF) is important in terms of both prognostication and treatment.In strictly WHO-defined ET,because the survival is near-normal,it would be inappropriate to suggest that any treatments modify the natural history of the disease.In contrast,the goal of current therapy in ET is primarily to prevent thrombohemorrhagic complications and secondarily to control the microcirculatory symptoms.In this regard,treatment is tailored to individual patients according to their risk for thrombosis or bleeding.Ruxolitinib is the first JAK inhibitor approved in the world with comfirmed clinical benefit to other MPNs,but the long-term efficacy of ruxolitinib treatment for ET is still lacking evidence.
Keywords：essential thrombocythemiapre-fibrotic/early primary myelofibrosisinternational prognostic scoreJAK inhibitor
Publication Date：2018-01-01
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：6( 98-103 )