title: Predictors of greater than 80% 2-year mortality in primary myelofibrosis: a Mayo Clinic study of 884 karyotypically annotated patients.
abstract: DIPSS-plus (the Dynamic International Prognostic Scoring System-plus) includes 8 risk factors for survival in primary myelofibrosis. In the present study of 884 karyotypically annotated patients with primary myelofibrosis, we sought to identify 1 or 2 parameters that can reliably predict death in the first 2 years of disease. After a median of 8.2 years from time of referral to the Mayo Clinic, 564 deaths (64% of patients in the study) had been recorded. Risk factors associated with > 80% 2-year mortality included monosomal karyotype, inv(3)/i(17q) abnormalities, or any 2 of the following: circulating blasts > 9%, leukocytes ≥ 40 × 10(9)/L, or other unfavorable karyotype. Patients with any 1 of these risk profiles (n = 52) displayed significantly shorter overall survival than those otherwise belonging to a high-risk category per DIPSS-plus (n = 298); respective median survivals were 9 and 23 months (hazard ratio 2.2, 95% confidence interval 1.6-3.1; P < .01). The present information complements DIPSS-plus in the selection of primary myelofibrosis patients for high-risk treatment approaches.
authors: Divisions of Hematology, Mayo Clinic, Rochester, MN, USA. tefferi.ayalew@mayo.edu
authors: Ayalew
authors: A
authors: Tefferi
authors: Thitina
authors: T
authors: Jimma
authors: Naseema
authors: N
authors: Gangat
authors: Rakhee
authors: R
authors: Vaidya
authors: Kebede H
authors: KH
authors: Begna
authors: Curtis A
authors: CA
authors: Hanson
authors: Daniel L
authors: DL
authors: Van Dyke
authors: Domenica
authors: D
authors: Caramazza
authors: Animesh
authors: A
authors: Pardanani
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002869:Chromosome Aberrations; D016208:Databases, Factual; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D059785:Karyotype; D007621:Karyotyping; D008297:Male; D008875:Middle Aged; D008910:Minnesota; D055728:Primary Myelofibrosis / Q000175:diagnosis* / Q000235:genetics / Q000401:mortality*; D011379:Prognosis; D012307:Risk Factors; D016019:Survival Analysis; D015996:Survival Rate; D013997:Time Factors; D055815:Young Adult