title: International Prognostic Scoring System-independent cytogenetic risk categorization in primary myelofibrosis.
abstract: Among 200 patients with primary myelofibrosis, karyotype at diagnosis was abnormal in 83 (42%). To assess their individual prognostic impact, specific cytogenetic abnormalities with more than or equal to 5 informative cases were identified and the rest grouped separately as "other abnormalities." Median survival in patients with sole +9 (n = 6), sole 20q- (n = 21), sole 13q- (n = 8), normal karyotype (n = 117), "other abnormalities" (n = 28), complex karyotype (n = 13), and sole +8 (n = 7) were "not reached," 112, 105, 80, 46, 34, and 28 months, respectively (P = .01). Accordingly, 4 cytogenetic risk groups were considered: (1) favorable (sole +9, 20q-, or 13q-), (2) normal, (3) unfavorable (complex karyotype or sole +8), and (4) "other abnormalities." Multivariable analysis confirmed the International Prognostic Scoring System (IPSS)-independent prognostic value of both 4-way and 2-way (ie, favorable/normal vs unfavorable/other abnormalities; IPSS-adjusted hazard ratio = 0.37; 95% confidence interval, 0.24-0.58) cytogenetic risk categorization (P < .01). The ability to prognostically dissect a specific IPSS category has major therapeutic implications.
authors.affiliation: Divisions of Hematology, Hematopathology, Mayo Clinic, Rochester, MN, USA.
authors.forename: Kebede
authors.initials: K
authors.lastname: Hussein
authors.forename: Animesh D
authors.initials: AD
authors.lastname: Pardanani
authors.forename: Daniel L
authors.initials: DL
authors.lastname: Van Dyke
authors.forename: Curtis A
authors.initials: CA
authors.lastname: Hanson
authors.forename: Ayalew
authors.initials: A
authors.lastname: Tefferi
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002869:Chromosome Aberrations / Q000706:statistics & numerical data; D003582:Cytogenetics / Q000379:methods*; D020022:Genetic Predisposition to Disease / Q000145:classification*; D005820:Genetic Testing / Q000379:methods; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007391:International Cooperation; D053614:Janus Kinase 2 / Q000235:genetics; D008875:Middle Aged; D009154:Mutation / Q000502:physiology; D055728:Primary Myelofibrosis / Q000175:diagnosis* / Q000235:genetics* / Q000401:mortality / Q000628:therapy; D011379:Prognosis; D012107:Research Design; D012306:Risk; D016019:Survival Analysis; D055815:Young Adult
chemical_list: D053614:Janus Kinase 2