title: The Dynamic International Prognostic Scoring System for myelofibrosis predicts outcomes after hematopoietic cell transplantation.
abstract: Studies by the International Working Group showed that the prognosis of myelofibrosis patients is predicted by the Dynamic International Prognostic Scoring System (DIPSS) risk categorization, which includes patient age, constitutional symptoms, hemoglobin, leukocyte count, and circulating blasts. We evaluated the prognostic usefulness of the DIPSS in 170 patients with myelofibrosis, 12 to 78 years of age (median, 51.5 years of age), who received hematopoietic cell transplantation (HCT) between 1990 and 2009 from related (n = 86) or unrelated donors (n = 84). By DIPSS, 21 patients had low-risk disease, 48 had intermediate-1, 50 had intermediate-2, and 51 had high-risk disease. Five-year incidence of relapse, relapse-free survival, overall survival, and nonrelapse mortality for all patients were 10%, 57%, 57%, and 34%, respectively. Among patients with DIPSS high-risk disease, the hazard ratio for post-HCT mortality was 4.11 (95% CI, 1.44-11.78; P = .008), and for nonrelapse mortality was 3.41 (95% CI, 1.15-10.09; P = .03) compared with low-risk patients. After a median follow-up of 5.9 years, the median survivals have not been reached for DIPSS risk groups low and intermediate-1, and were 7 and 2.5 years for intermediate-2 and high-risk patients, respectively. Thus, HCT was curative for a large proportion of patients with myelofibrosis, and post-HCT success was dependent on pre-HCT DIPSS classification.
authors.affiliation: Fred Hutchinson Cancer Research Center, Seattle, WA 98109-1024, USA. bscott@fhcrc.org
authors.forename: Bart L
authors.initials: BL
authors.lastname: Scott
authors.forename: Ted A
authors.initials: TA
authors.lastname: Gooley
authors.forename: Mohamed L
authors.initials: ML
authors.lastname: Sorror
authors.forename: Andrew R
authors.initials: AR
authors.lastname: Rezvani
authors.forename: Michael L
authors.initials: ML
authors.lastname: Linenberger
authors.forename: Jonathan
authors.initials: J
authors.lastname: Grim
authors.forename: Brenda M
authors.initials: BM
authors.lastname: Sandmaier
authors.forename: David
authors.initials: D
authors.lastname: Myerson
authors.forename: Thomas R
authors.initials: TR
authors.lastname: Chauncey
authors.forename: Rainer
authors.initials: R
authors.lastname: Storb
authors.forename: Veronika
authors.initials: V
authors.lastname: Buxhofer-Ausch
authors.forename: Jerald P
authors.initials: JP
authors.lastname: Radich
authors.forename: Frederick R
authors.initials: FR
authors.lastname: Appelbaum
authors.forename: H Joachim
authors.initials: HJ
authors.lastname: Deeg
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D005260:Female; D006454:Hemoglobins / Q000032:analysis; D006801:Humans; D008297:Male; D008875:Middle Aged; D015233:Models, Statistical; D009364:Neoplasm Recurrence, Local / Q000097:blood / Q000401:mortality* / Q000628:therapy*; D036102:Peripheral Blood Stem Cell Transplantation; D055728:Primary Myelofibrosis / Q000097:blood / Q000401:mortality* / Q000628:therapy*; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate; D055815:Young Adult
chemical_list: D006454:Hemoglobins