Update of Myeloproliferative Neoplasms

Polycytemia vera (PV) essential thrombocytosis(ET) and primary (PMFf) are categorizedby WHO as myeloproliferative neoplasms(MPN). There are clonal expansion of myeloid cells.jake2,CALR,and MPL are driver mutations that have important role in diagnosis andpathophysiology of these neoplasms .Polycythemia vera (PV) hase been diagnosed by presence of jake2 mutation andpolycythemia (Hb> 16.5 g/dl for male and 16 g/dl ). Bone marrow assessment is encouraged fordisringush PV from ET and obtain cytogenetic information. Current treatment only can increasedthrombosis free survival and leukemic fibrosis survival are not affected by treatment. Hydroxyureais the drug of choice if cytoreduction is needed .Essential thrombocytosis (ET) is diagnosed by platelet =450.000 /microL and presence ofjake2 ,CALR, MPL mutation or by exclusion of other causes of thrombocytosis. Bone marrowexamination should be done for distinguishing from PV and PMF.ET have the best prognosis inMPN and near normal survival controlling of thrombosis is the back bone of threatment in ET.Hydroxyurea is the first line treatment when cytoreduction is necessary.Primary myelofibrosis(PMF) is characterazwd by the presence of the driver mutation andclinical feature compatible with myelofibrosis. Bone marrow fibrosis might or might not have seenin PMF .two novel prognostic system for PMF were recently unveiled. GIPSS and MIPSS70 PMFhas the worst prognosis in MPN. Bone marrow transplantation may be used for high risk PMF.Asymptomatic low risk PMF may be follow and symptomatic patient may be treated according tosymptom.