The Dynamic International Prognostic Scoring System (DIPSS)-plus includes eight risk factors for survival in primary myelofibrosis (PMF). In the current study of 884 karyotypically-annotated patients with PMF, we sought to identify one or two parameters that can reliably predict death in the first two years of disease. After a median of 8.2 years from time of referral to the Mayo Clinic, 564 (64%) deaths have been recorded. Risk factors associated with >80% two-year mortality included monosomal karyotype, inv(3)/i(17q) abnormalities, or any two of the following: circulating blasts >9%, leukocytes ≥40 x 109/L, other unfavorable karyotype. Patients with any one of these risk profiles (n=52) displayed significantly shorter overall survival, compared to those otherwise belonging to high-risk category per DIPSS-plus (n=298); respective median survivals were 9 and 23 months (HR 2.2, 95% CI 1.6-3.1; p<0.01). The current information complements DIPSS-plus in the selection of PMF patients for high risk treatment approaches.

  • Submitted August 1, 2011.
  • Accepted August 22, 2011.