Pathogenesis and management of essential thrombocythemia.
The last four years have seen an explosion in our understanding of the myeloproliferative neoplasms. Important and often unexpected insights into the molecular mechanisms responsible for these disorders have been accompanied by the development of new diagnostic tests and by an improved understanding of the relationship between the different disease entities. This review will focus on recent developments in the pathogenesis and management of essential thrombocythemia with a particular emphasis on its phenotypic overlap with polycythemia vera and primary myelofibrosis.
Beer
Philip A
PA
Department of Haematology, University of Cambridge, Cambridge, United Kingdom.
Green
Anthony R
AR
D000208:Acute Disease; D000368:Aged; D001241:Aspirin / Q000627:therapeutic use; D002999:Clone Cells / Q000473:pathology; D019468:Disease Management; D018450:Disease Progression; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use; D016898:Interferon-alpha / Q000627:therapeutic use; D053614:Janus Kinase 2 / Q000037:antagonists & inhibitors / Q000235:genetics / Q000502:physiology; D007951:Leukemia, Myeloid / Q000473:pathology; D008875:Middle Aged; D008954:Models, Biological; D020125:Mutation, Missense; D011087:Polycythemia Vera / Q000175:diagnosis; D055728:Primary Myelofibrosis / Q000175:diagnosis; D011379:Prognosis; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use; D011799:Quinazolines / Q000627:therapeutic use; D013920:Thrombocythemia, Essential / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000235:genetics / Q000473:pathology; D019851:Thrombophilia / Q000188:drug therapy / Q000209:etiology
D016898:Interferon-alpha; D047428:Protein Kinase Inhibitors; D011799:Quinazolines; C507924:JAK2 protein, human; D053614:Janus Kinase 2; C021139:anagrelide; D001241:Aspirin; D006918:Hydroxyurea