Table 7.
MIPSS, MIPSS-plus, and GIPSS models for primary myelofibrosis
| Prognostic model and risk factors (weight) | Risk groups and median survival | |
|---|---|---|
| MIPSS70 | ||
| Genetic variables | • Clinical variables | |
| One HMR mutation (1 point) | Hemoglobin < 10 g/dL (1 point) | Low risk: 0–1 point (not reached) |
| ≥ 2 HMR mutations (2 points) | Leukocytes > 25 × 109/L (2 points) | Intermediate risk: 2–4 (6.3 yr) |
| Type 1/like CALR absent (1 point) | Platelet < 100 × 109/L (2 points) | High risk: ≥ 5 (3.1 yr) |
| Circulating blast ≥ 2% (1 point) | ||
| Constitutional symptom (1 point) | ||
| Marrow fibrosis grade ≥ 2 (1 point) | ||
| MIPSS70+ version 2.0 | ||
| • Genetic variables | • Clinical variables | |
| VHR karyotype (4 points) | Severe anemiaa (2 points) | Very low risk: 0 point (not reached) |
| Unfavorable karyotype (3 points) | Moderate anemiab (1 point) | Low risk: 1–2 (16.4 yr) |
| ≥ 2 HMR mutations (3 points) | Circulating blasts ≥ 2% (1 point) | Intermediate-1 risk: 3–4 (7.7 yr) |
| One HMR mutation (2 points) | Constitutional symptoms (2 points) | High risk: 5–8 (4.1 yr) |
| Type 1/like CALR absent (2 points) | Very high risk: ≥ 9 (1.8 yr) | |
| GIPSS | ||
| • Genetic variables | ||
| VHR karyotype (2 points) | Low risk: 0 point (26.4 yr) | |
| Unfavorable karyotype (1 point) | Intermediate-1 risk: 1 point (8 yr) | |
| Type 1/like CALR absent (1 point) | Intermediate-2 risk: 2 points (4.2 yr) | |
| ASXL1 mutation (1 point) | High risk: ≥ 3 points (2 yr) | |
| SRSF2 mutation (1 point) | ||
| U2AF1Q157 mutation (1 point) | ||
MIPSS70 for transplant-age patients (age ≤ 70 years); MIPSS70+ version 2.0: mutation and karyotype enhanced international prognostic system. Survival quotes are for age ≤ 70 years. Survival quotes are for all age groups; HMR mutations include ASXL1, SRSF2, EZH2, IDH1, IDH2, and in addition, for GIPSS and MIPSS70+ version 2.0, U2AF1Q157.
MIPSS, mutation-enhanced international prognostic scoring system; GIPSS, genetically inspired prognostic scoring system; HMR, high molecular risk; CALR, calreticulin; VHR, very high risk.
a
Severe anemia: Hemoglobin < 8 g/dL in women and < 9 g/dL in men.
b
Moderate anemia: Hemoglobin 8 to 9.9 in women and 9 to 10.9 in men.