title: It is time to change thrombosis risk assessment for PV and ET?
abstract: Polycythemia vera (PV) and essential thrombocythemia (ET) are myeloproliferative neoplasms to be diagnosed according to the WHO classification. Molecular profiling must include the analysis of JAK2 (looking for the V617F point-mutation in PV and ET, screening exon 12 for mutations only in V617F-negative PV), CALR and MPL mutations (both in V617F-negative ET). The current risk stratification to predict thrombosis requires two parameters: age over 60 years and prior history of thrombosis. On the basis of these two risk factors patients can be stratified in low-risk and high-risk and receive a proper treatment. However, a modern stratification of thrombotic risk might consider "new" low-risk patients: conventional low-risk plus absence of leukocytosis from diagnosis onwards and a hematocrit level below 45% during the course of disease for PV; conventional low-risk plus absence of leukocytosis from diagnosis onwards, JAK2 negativity, CALR positivity, and absence of cardiovascular risk factors for ET.
authors.affiliation: Division of Hematology, Department of Internal Medicine, University Hospital Ospedale di Circolo e Fondazione Macchi, Varese, Italy. Electronic address: francesco.passamonti@unipv.it.
authors.forename: Francesco
authors.initials: F
authors.lastname: Passamonti
authors.affiliation: Division of Hematology, Department of Internal Medicine, University Hospital Ospedale di Circolo e Fondazione Macchi, Varese, Italy. Electronic address: mimicaramazza@libero.it.
authors.forename: Domenica
authors.initials: D
authors.lastname: Caramazza
authors.affiliation: Division of Hematology, Department of Internal Medicine, University Hospital Ospedale di Circolo e Fondazione Macchi, Varese, Italy. Electronic address: barbara_mora@alice.it.
authors.forename: Barbara
authors.initials: B
authors.lastname: Mora
authors.affiliation: Genetic Unit, Medical Genetic and Cytogenetics Laboratory, SSD, SMEL, University Hospital Ospedale di Circolo e Fondazione Macchi, Varese, Italy. Electronic address: rosario.casalone@ospedale.varese.it.
authors.forename: Rosario
authors.initials: R
authors.lastname: Casalone
authors.affiliation: Division of Hematology, Department of Internal Medicine, University Hospital Ospedale di Circolo e Fondazione Macchi, Varese, Italy. Electronic address: margherita.maffioli@gmail.com.
authors.forename: Margherita
authors.initials: M
authors.lastname: Maffioli
mesh_terms: D000367:Age Factors; D000368:Aged; D000970:Antineoplastic Agents / Q000627:therapeutic use; D037282:Calreticulin / Q000235:genetics*; D005260:Female; D006400:Hematocrit; D006801:Humans; D053614:Janus Kinase 2 / Q000235:genetics*; D008297:Male; D008875:Middle Aged; D009154:Mutation; D011087:Polycythemia Vera / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D011379:Prognosis; D053628:Receptors, Thrombopoietin / Q000235:genetics*; D018570:Risk Assessment; D013920:Thrombocythemia, Essential / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D013927:Thrombosis / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics / Q000473:pathology
chemical_list: D000970:Antineoplastic Agents; D037282:Calreticulin; D053628:Receptors, Thrombopoietin; C082218:MPL protein, human; C507924:JAK2 protein, human; D053614:Janus Kinase 2
keywords: CALR; JAK2; polycythemia; prognosis; thrombocythemia; thrombosis