title: Risk models predicting survival after reduced-intensity transplantation for myelofibrosis.
abstract: To define a prognostic model for predicting outcome of reduced-intensity allogeneic stem cell transplantation (RIC-ASCT) for myelofibrosis we evaluated 150 homogeneously treated patients and developed a new risk score for overall survival (OS). In a multivariate Cox model for OS, only JAK2 V617F wild-type, age ≥57 years and constitutional symptoms were independently predictive for OS (Hazard Ratio: 2·02; 2·43 and 2·80 respectively). Depending on the presence of one, two or all of these factors, HR of death was 3·08; 4·70 and 16·61 respectively (P < 0·001). This score was compared to the Lille, Cervantes, International Prognostic Scoring System (IPSS), dynamic IPSS (DIPSS) and modified European Blood and Marrow Transplantation Group (EBMT) scores. Lille score correlated significantly with OS but discriminated poorly between the intermediate and high-risk groups (5-year OS 56% and 51% respectively). IPSS and DIPSS correlated significantly with OS but differences between intermediate-1 and intermediate-2 groups were not significant (5-year OS 78% vs. 78% and 70%, 60% respectively). Modified EBMT and Cervantes models did not predict OS post-ASCT. In conclusion, a simple model which includes: age, JAK2 V617F-status and constitutional symptoms can clearly separate distinct risk groups and can be used in addition to the Lille model to predict OS after RIC-ASCT for myelofibrosis.
authors.affiliation: Department of Stem Cell Transplantation, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany.
authors.forename: Haefaa
authors.initials: H
authors.lastname: Alchalby
authors.forename: Dinah-Rohina
authors.initials: DR
authors.lastname: Yunus
authors.forename: Tatjana
authors.initials: T
authors.lastname: Zabelina
authors.forename: Guido
authors.initials: G
authors.lastname: Kobbe
authors.forename: Ernst
authors.initials: E
authors.lastname: Holler
authors.forename: Martin
authors.initials: M
authors.lastname: Bornhäuser
authors.forename: Rainer
authors.initials: R
authors.lastname: Schwerdtfeger
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Bethge
authors.forename: Hans Michael
authors.initials: HM
authors.lastname: Kvasnicka
authors.forename: Guntram
authors.initials: G
authors.lastname: Büsche
authors.forename: Francis
authors.initials: F
authors.lastname: Ayuk
authors.forename: Ulrike
authors.initials: U
authors.lastname: Bacher
authors.forename: Axel R
authors.initials: AR
authors.lastname: Zander
authors.forename: Nicolaus
authors.initials: N
authors.lastname: Kröger
mesh_terms: D000328:Adult; D000368:Aged; D019943:Amino Acid Substitution; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D053614:Janus Kinase 2 / Q000235:genetics / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D008954:Models, Biological; D020125:Mutation, Missense; D055728:Primary Myelofibrosis / Q000201:enzymology / Q000235:genetics / Q000401:mortality* / Q000628:therapy*; D018570:Risk Assessment; D033581:Stem Cell Transplantation; D015996:Survival Rate; D013997:Time Factors; D019172:Transplantation Conditioning; D014184:Transplantation, Homologous
chemical_list: C507924:JAK2 protein, human; D053614:Janus Kinase 2