Back to Search
Start Over
New prognostic scoring system for primary myelofibrosis based on a study of the International Working Group for Myelofibrosis Research and Treatment
- Publication Year :
- 2009
-
Abstract
- Therapeutic decision-making in primary myelofibrosis (PMF) is becoming more challenging because of the increasing use of allogeneic stem cell transplantation and new investigational drugs. To enhance this process by developing a highly discriminative prognostic system, 1054 patients consecutively diagnosed with PMF at 7 centers were studied. Overall median survival was 69 months (95% confidence interval [CI]: 61-76). Multivariate analysis of parameters obtained at disease diagnosis identified age greater than 65 years, presence of constitutional symptoms, hemoglobin level less than 10 g/dL, leukocyte count greater than 25 × 109/L, and circulating blast cells 1% or greater as predictors of shortened survival. Based on the presence of 0 (low risk), 1 (intermediate risk-1), 2 (intermediate risk-2) or greater than or equal to 3 (high risk) of these variables, 4 risk groups with no overlapping in their survival curves were delineated; respective median survivals were 135, 95, 48, and 27 months (P < .001). Compared with prior prognostic models, the new risk stratification system displayed higher predictive accuracy, replicability, and discriminating power. In 409 patients with assessable metaphases, cytogenetic abnormalities were associated with shorter survival, but their independent contribution to prognosis was restricted to patients in the intermediate-risk groups. JAK2V617F did not cluster with a specific risk group or affect survival.
- Subjects :
- Oncology
Adult
Male
Ruxolitinib
medicine.medical_specialty
Palliative care
Adolescent
Constitutional symptoms
International Cooperation
Immunology
acute myeloid leukemia
Biochemistry
survival
Young Adult
Internal medicine
Cause of Death
medicine
Humans
Myelofibrosis
Child
Survival analysis
thrombosis
Aged
Aged, 80 and over
business.industry
Cell Biology
Hematology
Congresses as Topic
Middle Aged
medicine.disease
Survival Analysis
Confidence interval
Surgery
Transplantation
Myeloproliferative disorders
Pacritinib
JAK2
Research Design
primary myelofibrosis
Female
prognosis
business
medicine.drug
Subjects
Details
- Language :
- English
- Database :
- OpenAIRE
- Accession number :
- edsair.doi.dedup.....aac27cdad62f26b080f0fdee9c188abd