Table 1.
Summary table for the three most recent prognostic models in primary myelofibrosis
| MIPSS70 (3-tiered) | MIPSS70+ version 2.0 (5-tiered) | GIPSS (4-tiered) | |||
|---|---|---|---|---|---|
|
Genetic variables: One HMR mutation (1 point) ≥2 HMR mutations (2 points) Type 1/like CALR absent (1 point) |
Clinical variables: Hemoglobin <10 g/dl (1 point) Leukocytes >25 × 109/l (2 points) Platelets < 100 × 109/l (2 points) Circulating blasts ≥2% (1 point) Constitutional symptoms (1 point) Bone marrow fibrosis grade ≥2 (1 point) |
Genetic variables:
VHR karyotype (4 points) Unfavorable karyotype (3 points) ≥2 HMR mutations (3 points) One HMR mutation (2 points) Type 1/like CALR absent (2 points) |
Clinical variables:
Severe anemia (2 points) Moderate anemia (1 point) Circulating blasts ≥2% (1 point) Constitutional symptoms (2 points) |
Genetic variables: VHR karyotype (2 points) Unfavorable karyotype (1 point) Type 1/like CALR absent (1 point) ASXL1 mutation (1 point) SRSF2 mutation (1 point) U2AF1Q157 mutation (1 point) |
|
| Very low risk (median survival) | Zero points (not reached) | ||||
| Low risk (median survival) | 0–1 points (not reached) | 1–2 points (16.4 years) | Zero points (26.4 years) | ||
| Intermediate-1 risk (median survival) | One point (8 years) | ||||
| Intermediate risk (median survival) | 2–4 points (6.3 years) | 3–4 points (7.7 years) | |||
| Intermediate-2 risk (median survival) | Two points (4.2 years) | ||||
| High risk (median survival) | ≥5 points (3.1 years) | 5–8 points (4.1 years) | ≥3 points (2 years) | ||
| Very high risk (median survival) | ≥9 points (1.8 years) | ||||
MIPSS70: mutation-enhanced international prognostic system for transplant-age patients (age ≤70 years)33
MIPSS70+ version 2.0: mutation and karyotype enhanced international prognostic system (J. Clin. Oncol. 2018. doi: 10.1200/JCO.2018.78.9867). Survival quotes are for age ≤70 years
GIPSS: genetically inspired prognostic scoring system19. Survival quotes are for all age groups
HMR: high molecular risk mutations include ASXL1, SRSF2, EZH2, IDH1, IDH2 and, in addition, for GIPSS and MIPSS70+ version 2.0, U2AF1Q157
VHR: very high-risk karyotype
Severe anemia: Hemoglobin <8 g/dl in women and <9 g/dl in men
Moderate anemia: Hemoglobin 8–9.9 in women and 9–10.9 in men