PURPOSE OF REVIEW: Management of @entity6582 vera and @entity4407 requires understanding of the key concepts regarding diagnosis, risk stratification, and management. RECENT FINDINGS: Essential @entity1148 and @entity6582 vera are among the Philadelphia chromosome negative @entity5 . They are characterized by overproduction of blood cells and their complications include @entity715 , @entity548 , and progression to @entity4018 or @entity3052 ( @entity3052 ). Management of @entity4406 requires recognition of the risk factors for @entity715 and @entity548 . Risk stratification allows the clinician to make a treatment plan that may include antiplatelet therapy with @entity117 alone or in combination with therapeutic phlebotomy in the case of @entity6582 vera, or cytoreductive therapy for high-risk @entity1 with either @entity4407 or @entity6582 vera. Hydroxyurea remains first-line therapy for high-risk @entity1 with @entity4406 , whereas second-line options include @entity18829 , pegylated-IFNa-2a, and the @entity7821 inhibitor @entity26223 . The current evaluation of pegylated- @entity4020 a in global phase II and III studies will provide clarity to the potential long-term benefit and risks associated with this biologic in @entity1 with @entity4406 . Novel therapeutics aimed at prevention of disease progression to @entity4018 / @entity3052 are the focus of current clinical trials. SUMMARY: Risk stratification of @entity1 with @entity4406 by age and/or history of @entity715 provides the basis of risk adapted therapeutic intervention. Aggressive control of modifiable cardiovascular risk factors, the use of antiplatelet agents, control of the hematocrit less than 45% in @entity6582 vera, and cytoreductive therapy in high-risk @entity4406 @entity1 is the focus of management. The exact role of @entity4020 remains undefined and under active investigation, and the recent approval of @entity26223 provides @entity1 with @entity6582 vera a second-line option.

Contemporary approach to @entity4407 and XXXX vera.

@entity1 :: ('9606', 'Species') :: ['patients'] @entity6582 :: ('MESH:D011086', 'Disease') :: ['polycythemia'] @entity1148 :: ('MESH:D013922', 'Disease') :: ['thrombocythemia'] @entity4020 :: ('3440', 'Gene') :: ['IFN-a', 'IFNa-2'] @entity548 :: ('MESH:D006470', 'Disease') :: ['hemorrhage'] @entity7821 :: ('3716;3717', 'Gene') :: ['JAK1/2'] @entity5 :: ('MESH:D009369', 'Disease') :: ['myeloproliferative neoplasms'] @entity117 :: ('MESH:D001241', 'Chemical') :: ['aspirin'] @entity26223 :: ('MESH:C540383', 'Chemical') :: ['ruxolitinib'] @entity3052 :: ('MESH:D015470', 'Disease') :: ['acute myeloid leukemia', 'AML'] @entity4018 :: ('MESH:D055728', 'Disease') :: ['myelofibrosis'] @entity4406 :: ('MESH:D011087', 'Disease') :: ['essential thrombocythemia/polycythemia vera'] @entity4407 :: ('MESH:D013920', 'Disease') :: ['essential thrombocythemia'] @entity18829 :: ('MESH:C021139', 'Chemical') :: ['anagrelide'] @entity715 :: ('MESH:D013927', 'Disease') :: ['thrombosis']
@entity6582 :: (MESH:D011086,Disease) :: ['polycythemia']
