title: Therapy with JAK2 inhibitors for myeloproliferative neoplasms.
abstract: The development of JAK2 inhibitors followed the discovery of activating mutation of JAK2 (JAK2V617F) in patients with classic Philadelphia-negative myeloproliferative neoplasms (Ph-negative MPN). It is now known that mutations activating the JAK-STAT pathway are ubiquitous in Ph-negative MPN, and that the deregulated JAK-STAT pathway plays a central role in the pathogenesis of these disorders. JAK2 inhibitors thus are effective in patients both with and without the JAK2V617F mutation. This article reviews the rationale for using JAK2 inhibitors in Ph-negative MPN, and the results of more recent clinical trials with these drugs.
authors.affiliation: Hematology and Oncology Center, Hospital Israelita Albert Einstein, Avenida Albert Einstein, 627/701, Building A, Sao Paulo, SP 05651-901, Brazil.
authors.forename: Fabio P S
authors.initials: FP
authors.lastname: Santos
authors.forename: Srdan
authors.initials: S
authors.lastname: Verstovsek
mesh_terms: D006801:Humans; D053614:Janus Kinase 2 / Q000037:antagonists & inhibitors*; D009196:Myeloproliferative Disorders / Q000188:drug therapy* / Q000201:enzymology; D011379:Prognosis; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use*
chemical_list: D047428:Protein Kinase Inhibitors; C507924:JAK2 protein, human; D053614:Janus Kinase 2