title: Philadelphia-negative classical myeloproliferative neoplasms: critical concepts and management recommendations from European LeukemiaNet.
abstract: We present a review of critical concepts and produce recommendations on the management of Philadelphia-negative classical myeloproliferative neoplasms, including monitoring, response definition, first- and second-line therapy, and therapy for special issues. Key questions were selected according the criterion of clinical relevance. Statements were produced using a Delphi process, and two consensus conferences involving a panel of 21 experts appointed by the European LeukemiaNet (ELN) were convened. Patients with polycythemia vera (PV) and essential thrombocythemia (ET) should be defined as high risk if age is greater than 60 years or there is a history of previous thrombosis. Risk stratification in primary myelofibrosis (PMF) should start with the International Prognostic Scoring System (IPSS) for newly diagnosed patients and dynamic IPSS for patients being seen during their disease course, with the addition of cytogenetics evaluation and transfusion status. High-risk patients with PV should be managed with phlebotomy, low-dose aspirin, and cytoreduction, with either hydroxyurea or interferon at any age. High-risk patients with ET should be managed with cytoreduction, using hydroxyurea at any age. Monitoring response in PV and ET should use the ELN clinicohematologic criteria. Corticosteroids, androgens, erythropoiesis-stimulating agents, and immunomodulators are recommended to treat anemia of PMF, whereas hydroxyurea is the first-line treatment of PMF-associated splenomegaly. Indications for splenectomy include symptomatic portal hypertension, drug-refractory painful splenomegaly, and frequent RBC transfusions. The risk of allogeneic stem-cell transplantation-related complications is justified in transplantation-eligible patients whose median survival time is expected to be less than 5 years.
authors.affiliation: Unit of Clinical Epidemiology/Center for the Study of Myelofibrosis, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Policlinico S. Matteo, Viale Golgi 19, 27100 Pavia, Italy.
authors.forename: Tiziano
authors.initials: T
authors.lastname: Barbui
authors.forename: Giovanni
authors.initials: G
authors.lastname: Barosi
authors.forename: Gunnar
authors.initials: G
authors.lastname: Birgegard
authors.forename: Francisco
authors.initials: F
authors.lastname: Cervantes
authors.forename: Guido
authors.initials: G
authors.lastname: Finazzi
authors.forename: Martin
authors.initials: M
authors.lastname: Griesshammer
authors.forename: Claire
authors.initials: C
authors.lastname: Harrison
authors.forename: Hans Carl
authors.initials: HC
authors.lastname: Hasselbalch
authors.forename: Rudiger
authors.initials: R
authors.lastname: Hehlmann
authors.forename: Ronald
authors.initials: R
authors.lastname: Hoffman
authors.forename: Jean-Jacques
authors.initials: JJ
authors.lastname: Kiladjian
authors.forename: Nicolaus
authors.initials: N
authors.lastname: Kröger
authors.forename: Ruben
authors.initials: R
authors.lastname: Mesa
authors.forename: Mary F
authors.initials: MF
authors.lastname: McMullin
authors.forename: Animesh
authors.initials: A
authors.lastname: Pardanani
authors.forename: Francesco
authors.initials: F
authors.lastname: Passamonti
authors.forename: Alessandro M
authors.initials: AM
authors.lastname: Vannucchi
authors.forename: Andreas
authors.initials: A
authors.lastname: Reiter
authors.forename: Richard T
authors.initials: RT
authors.lastname: Silver
authors.forename: Srdan
authors.initials: S
authors.lastname: Verstovsek
authors.forename: Ayalew
authors.initials: A
authors.lastname: Tefferi
mesh_terms: D006801:Humans; D054438:Leukemia, Myeloid, Chronic, Atypical, BCR-ABL Negative / Q000628:therapy*