Myelofibrosis
Prognostic score: International working group for myelofibrosis (at diagnosis)
- Age > 65
- Constitutional symptoms
- Hgb < 100 g/l
- WBC > 25
- Blasts in peripheral blood > 1%
Risk group | No. of factors | Median survival (mo; 95% CI) | Proportion of deaths, % |
---|---|---|---|
Low | 0 | 135 (117-181) | 32 |
Intermediate-1 | 1 | 95 (79-114) | 50 |
Intermediate-2 | 2 | 48 (43-59) | 71 |
High | > 3 | 27 (23-31) | 73 |
The presence of an abnormal karyotype contributes to a worse prognosis only in the intermediate risk group (1 and 2).
Prognostic factors: At any time during follow up
Factors associated with a poor prognosis at diagnosis or during follow up ; each of these factors is associated with a median survival of less than 12 months :
- Blasts in blood or marrow ≥ 10%: median survival 10 months
- Platelets ≤ 50: median survival 12 months
- Abnormalities of chromosome 17: median survival 5 months
Indications for allogeneic transplant
We recommend proceeding with transplant in the intermediate 2 and high risk categories up until the ages of 65 with a related donor and until 60 with an unrelated donor.
A patient that has any of the 3 poor prognostic features listed above becomes eligible regardless of the risk category.
In certain situations, an allogeneic transplant will be considered in younger patients in the intermediate 1 risk category if an additional poor prognostic feature is present (example: transfusion dependence not responding to other therapies).
