title: The European Consensus on grading of bone marrow fibrosis allows a better prognostication of patients with primary myelofibrosis.
abstract: We investigated the relationship between the International Prognostic Scoring System of the International Working Group for Myelofibrosis Research and Treatment and the European Consensus on grading of bone marrow fibrosis (MF) in patients with primary myelofibrosis. We compared them in 196 consecutive primary myelofibrosis patients (median follow-up 45.7 months; range 7.4-159). International Prognostic Scoring System classified 42 cases as low risk, 73 as intermediate risk-1, 69 as intermediate risk-2, and 12 as high risk; European Consensus on grading of bone marrow fibrosis classified 83 cases as MF-0, 58 as MF-1, 41 as MF-2, and 14 as MF-3. By the time of the analysis, 30 patients (15.3%) had died. Overall median survival was 3.8 years (95% confidence interval: 3.3-4.3). Multivariate analysis confirmed that both scoring systems independently predicted survival, with hazard ratios similar to those provided by univariate analysis (respectively, 2.40 (95% confidence interval: 1.47-3.91) and 2.58 (95% confidence interval: 1.72-3.89) but the likelihood ratio increased from 19.6 of the International Prognostic Scoring System or 29.0 of the European Consensus on grading of bone MF to 42.3 when both measures were considered together. Analysis of the overall survival curves documented that patients classified as having the most favourable rate with both prognostic scores (ie low risk and MF-0) survive longer than those with only one favourable score (ie low risk but MF >0 or MF-0, but International Prognostic Scoring System >low risk). In contrast, those patients classified as having the most unfavourable rate for both scores (high risk and MF-3) have a shorter survival than those with only one unfavourable score (ie high risk but MF<3 or MF-3, but International Prognostic Scoring System <high risk). In conclusion, our analysis suggests that better prognostication can be achieved in primary myelofibrosis patients when both systems are used together.
authors.affiliation: U.O.C di Anatomia Patologica, Università degli Studi di Milano, Dipartimento di Medicina, Chirurgia e Odontoiatria, Fondazione IRCCS Ca' Granda-Ospedale Maggiore Policlinico, Milan, Italy. umberto.gianelli@unimi.it
authors.forename: Umberto
authors.initials: U
authors.lastname: Gianelli
authors.forename: Claudia
authors.initials: C
authors.lastname: Vener
authors.forename: Anna
authors.initials: A
authors.lastname: Bossi
authors.forename: Ivan
authors.initials: I
authors.lastname: Cortinovis
authors.forename: Alessandra
authors.initials: A
authors.lastname: Iurlo
authors.forename: Nicola S
authors.initials: NS
authors.lastname: Fracchiolla
authors.forename: Federica
authors.initials: F
authors.lastname: Savi
authors.forename: Alessia
authors.initials: A
authors.lastname: Moro
authors.forename: Federica
authors.initials: F
authors.lastname: Grifoni
authors.forename: Chiara
authors.initials: C
authors.lastname: De Philippis
authors.forename: Tommaso
authors.initials: T
authors.lastname: Radice
authors.forename: Silvano
authors.initials: S
authors.lastname: Bosari
authors.forename: Giorgio
authors.initials: G
authors.lastname: Lambertenghi Deliliers
authors.forename: Agostino
authors.initials: A
authors.lastname: Cortelezzi
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001853:Bone Marrow / Q000473:pathology*; D003245:Consensus Statements as Topic; D005260:Female; D005355:Fibrosis; D006801:Humans; D007391:International Cooperation; D007558:Italy / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D055728:Primary Myelofibrosis / Q000145:classification / Q000401:mortality / Q000473:pathology*; D011379:Prognosis; D015996:Survival Rate