Modern risk stratification systems for survival and leukemic transformation in primary myelofibrosis
| Risk stratification system . | Applicability . | Prognostic factors . | Risk score . | Median survival, mo . | Comments . |
|---|---|---|---|---|---|
| IPSS4 | Diagnosis | Age > 65 y | 1 | Low risk (0), 135 | |
| Anemia (Hb < 10 g/dL) | 1 | Intermediate 1 risk (1), 95 | |||
| WBC count > 25 × 109/L | 1 | Intermediate 2 risk (2), 48 | |||
| Blood blasts > 1% | 1 | High ( ≥ 3), 27 | |||
| Constitutional symptoms | 1 | ||||
| DIPSS6 | Any time in disease course | Age > 65 y | 1 | Low risk (0), not reached | DIPSS also predicts evolution to AML, Hazard ratios of 7.8 and 24.9 for Intermediate-2 and high-risk category compared with low risk23 |
| Anemia (Hb < 10 g/dL) | 2 | Intermediate 1 risk (1-2), 170 | |||
| WBC count > 25 × 109/L | 1 | Intermediate 2 risk (3-4), 48 | |||
| Blood blasts ≥ 1% | 1 | High (5-6), 18 | |||
| Constitutional symptoms | 1 | ||||
| DIPSS plus5 | Any time in disease course | DIPSS low risk | 0 | Low risk (0), 185 | First calculate DIPSS score6 and then add the score of transfusion dependency, cytogenetics, and thrombocytopenia to calculate final DIPSS plus score |
| DIPSS intermediate-1 | 1 | Intermediate 1 risk (1), 78 | |||
| DIPSS intermediate-2 | 2 | Intermediate 2 risk (2-3), 35 | |||
| DIPSS high-risk | 3 | High (≥ 4), 16 | Based on single institution data, needs validation | ||
| PLUS | |||||
| Transfusion dependency | 1 | ||||
| Unfavorable cytogenetics* | 1 | ||||
| Platelets < 100 × 109/L | 1 | ||||
| IWG risk model for LT25 | Any time in disease course | Risk factors: high-risk cytogenetics† | 2 | Risk of LT: low (score 0), 3% at 3 y | |
| Risk factors: blood blasts ≥ 2% | 1 | Risk of LT: intermediate (score 1), 10% at 3 y | |||
| Risk factors: platelets ≤ 50 × 109/L | 1 | Risk of LT: high (score 2), 35% at 3 y |
| Risk stratification system . | Applicability . | Prognostic factors . | Risk score . | Median survival, mo . | Comments . |
|---|---|---|---|---|---|
| IPSS4 | Diagnosis | Age > 65 y | 1 | Low risk (0), 135 | |
| Anemia (Hb < 10 g/dL) | 1 | Intermediate 1 risk (1), 95 | |||
| WBC count > 25 × 109/L | 1 | Intermediate 2 risk (2), 48 | |||
| Blood blasts > 1% | 1 | High ( ≥ 3), 27 | |||
| Constitutional symptoms | 1 | ||||
| DIPSS6 | Any time in disease course | Age > 65 y | 1 | Low risk (0), not reached | DIPSS also predicts evolution to AML, Hazard ratios of 7.8 and 24.9 for Intermediate-2 and high-risk category compared with low risk23 |
| Anemia (Hb < 10 g/dL) | 2 | Intermediate 1 risk (1-2), 170 | |||
| WBC count > 25 × 109/L | 1 | Intermediate 2 risk (3-4), 48 | |||
| Blood blasts ≥ 1% | 1 | High (5-6), 18 | |||
| Constitutional symptoms | 1 | ||||
| DIPSS plus5 | Any time in disease course | DIPSS low risk | 0 | Low risk (0), 185 | First calculate DIPSS score6 and then add the score of transfusion dependency, cytogenetics, and thrombocytopenia to calculate final DIPSS plus score |
| DIPSS intermediate-1 | 1 | Intermediate 1 risk (1), 78 | |||
| DIPSS intermediate-2 | 2 | Intermediate 2 risk (2-3), 35 | |||
| DIPSS high-risk | 3 | High (≥ 4), 16 | Based on single institution data, needs validation | ||
| PLUS | |||||
| Transfusion dependency | 1 | ||||
| Unfavorable cytogenetics* | 1 | ||||
| Platelets < 100 × 109/L | 1 | ||||
| IWG risk model for LT25 | Any time in disease course | Risk factors: high-risk cytogenetics† | 2 | Risk of LT: low (score 0), 3% at 3 y | |
| Risk factors: blood blasts ≥ 2% | 1 | Risk of LT: intermediate (score 1), 10% at 3 y | |||
| Risk factors: platelets ≤ 50 × 109/L | 1 | Risk of LT: high (score 2), 35% at 3 y |