title: Initial bone marrow reticulin fibrosis in polycythemia vera exerts an impact on clinical outcome.
abstract: We examined the prevalence and prognostic relevance of bone marrow reticulin fibrosis in 526 patients with World Health Organization-defined polycythemia vera evaluated at the time of initial diagnosis. Seventy-four patients (14%) displayed mostly grade 1 reticulin fibrosis, with only 2 cases showing higher-grade fibrosis. Presenting clinical and laboratory characteristics, including JAK2V617F allele burden, between patients with and without fibrosis were similar for the most part, with the exception of a higher prevalence of palpable splenomegaly in patients with fibrosis (P < .01). Patients with fibrosis were less prone to experience thrombosis during their clinical course (1.1 vs 2.7 per 100 patient-years; P = .03) and more prone to develop post-polycythemia vera myelofibrosis (2.2 vs 0.8 per 100 patient-years; P = .01). There was no significant difference between the 2 groups in terms of overall or leukemia-free survival. The present study clarifies the incidence, degree, and prognostic relevance of bone marrow fibrosis obtained at time of initial diagnosis of polycythemia vera.
authors.affiliation: Ospedali Riuniti di Bergamo, Italy. tbarbui@ospedaliriuniti.bergamo.it
authors.forename: Tiziano
authors.initials: T
authors.lastname: Barbui
authors.forename: Jürgen
authors.initials: J
authors.lastname: Thiele
authors.forename: Francesco
authors.initials: F
authors.lastname: Passamonti
authors.forename: Elisa
authors.initials: E
authors.lastname: Rumi
authors.forename: Emanuela
authors.initials: E
authors.lastname: Boveri
authors.forename: Maria Luigia
authors.initials: ML
authors.lastname: Randi
authors.forename: Irene
authors.initials: I
authors.lastname: Bertozzi
authors.forename: Filippo
authors.initials: F
authors.lastname: Marino
authors.forename: Alessandro M
authors.initials: AM
authors.lastname: Vannucchi
authors.forename: Lisa
authors.initials: L
authors.lastname: Pieri
authors.forename: Giada
authors.initials: G
authors.lastname: Rotunno
authors.forename: Heinz
authors.initials: H
authors.lastname: Gisslinger
authors.forename: Bettina
authors.initials: B
authors.lastname: Gisslinger
authors.forename: Leonhard
authors.initials: L
authors.lastname: Müllauer
authors.forename: Guido
authors.initials: G
authors.lastname: Finazzi
authors.forename: Alessandra
authors.initials: A
authors.lastname: Carobbio
authors.forename: Andrea
authors.initials: A
authors.lastname: Gianatti
authors.forename: Marco
authors.initials: M
authors.lastname: Ruggeri
authors.forename: Ilaria
authors.initials: I
authors.lastname: Nichele
authors.forename: Emanuele
authors.initials: E
authors.lastname: D'Amore
authors.forename: Alessandro
authors.initials: A
authors.lastname: Rambaldi
authors.forename: Ayalew
authors.initials: A
authors.lastname: Tefferi
mesh_terms: D001853:Bone Marrow / Q000378:metabolism / Q000473:pathology*; D018450:Disease Progression; D005355:Fibrosis; D005500:Follow-Up Studies; D006801:Humans; D053614:Janus Kinase 2 / Q000235:genetics; D007938:Leukemia / Q000150:complications; D009154:Mutation; D011087:Polycythemia Vera / Q000150:complications / Q000235:genetics / Q000473:pathology*; D055728:Primary Myelofibrosis / Q000150:complications; D011379:Prognosis; D012155:Reticulin / Q000378:metabolism; D016019:Survival Analysis; D013927:Thrombosis / Q000150:complications
chemical_list: D012155:Reticulin; C507924:JAK2 protein, human; D053614:Janus Kinase 2