title: Update from the latest WHO classification of MPNs: a user's manual.
abstract: The 2016 multiparameter World Health Organization (WHO) classification for Philadelphia-negative myeloproliferative neoplasms (MPNs) integrates clinical features, morphology, and genetic data to diagnose polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). The main novelties are: (1) the reduction of the hemoglobin (Hb) level threshold to diagnose PV, now established at 16.5 g/dL for men and 16 g/dL for women (based on the identification of MPN patients with PV-consistent bone marrow [BM] features and a Hb level lower than that established in the 2008 WHO classification for PV); (2) the recognition of prefibrotic/early PMF, distinguishable from ET on the basis of BM morphology, an entity having a higher tendency to develop overt myelofibrosis or acute leukemia, and characterized by inferior survival; (3) the central role of BM morphology in the diagnosis of ET, prefibrotic/early PMF, PMF, and PV with borderline Hb values; megakaryocyte number and morphology (typical in ET, atypical in both PMF forms) accompanied by a new distinction of reticulin fibrosis grade in PMF (grade 1 in prefibrotic/early PMF and grade 2-3 in PMF) constitute diagnostic criteria; and (4) the inclusion of all mutually exclusive MPN driver mutations (JAK2, CALR, and MPL) as major diagnostic criteria in ET and PMF; 10% to 15% of these patients are triple negative, and in these cases the search for an additional clonal marker (eg, mutations in ASXL1, EZH2, TET2, IDH1/IDH2, SRSF2, and SF3B1) is warranted.
authors.affiliation: Department of Experimental and Clinical Medicine, University of Insubria, Varese, Italy; and.|Hematology, Department of Specialistic Medicine, Ospedale di Circolo, Azienda Socio Sanitaria Territoriale Sette Laghi, Varese, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Passamonti
authors.affiliation: Hematology, Department of Specialistic Medicine, Ospedale di Circolo, Azienda Socio Sanitaria Territoriale Sette Laghi, Varese, Italy.
authors.forename: Margherita
authors.initials: M
authors.lastname: Maffioli
mesh_terms: D005260:Female; D019337:Hematologic Neoplasms / Q000145:classification* / Q000175:diagnosis* / Q000628:therapy; D006801:Humans; D008297:Male; D009154:Mutation; D009196:Myeloproliferative Disorders / Q000145:classification* / Q000175:diagnosis* / Q000628:therapy; D009363:Neoplasm Proteins / Q000097:blood / Q000235:genetics; D014944:World Health Organization
chemical_list: D009363:Neoplasm Proteins