Table 1.
Brief summary of all the prognostic models mentioned above that are actually used worldwide, with a demonstration of the explanation of the scores assigned to each variable and a description of the risk categories identified with their corresponding median overall survival. BM: bone marrow; HMR: high-molecular-risk; VHR: very-high-risk; RUX: ruxolitinib; RBC: red blood cells; NR: not-reached; OS: overall-survival.
| Prognostic Model | Variable | Points | Risk Category |
Score | Median Survival (Years) |
|---|---|---|---|---|---|
| IPSS | Constitutional symptoms | 1 | LOW | 0 | 11.3 |
| Hemoglobin < 10 g/dL | 1 | INTERMEDIATE-1 | 1 | 7.9 | |
| WBC > 25 × 109/L | 1 | INTERMEDIATE-2 | 2 | 4 | |
| Blast ≥ 1% | 1 | HIGH | ≥3 | 2.3 | |
| Age > 65 | 1 | / | / | / | |
| DIPSS | Constitutional symptoms | 1 | LOW | 0 | NR |
| Hemoglobin < 10 g/dL | 2 | INTERMEDIATE-1 | 1–2 | 14.2 | |
| WBC > 25 × 109/L | 1 | INTERMEDIATE-2 | 3–4 | 4 | |
| Blast ≥ 1% | 1 | HIGH | ≥5 | 1.5 | |
| Age > 65 | 1 | / | / | / | |
| DIPSS-Plus | DIPSS added to | LOW | 0 | 15 | |
| transfusion requirement | 1 | INTERMEDIATE-1 | 1 | 6.7 | |
| Platelet count < 100 × 109/L | 1 | INTERMEDIATE-2 | 2–3 | 2.9 | |
| Unfavorable karyotype | 1 | HIGH | ≥4 | 1.3 | |
| MYSEC-PM | Age | 0.15 per year | LOW | <11 | NR |
| Hemoglobin < 11 g/d | 2 | INTERMEDIATE 1 | ≥11, <14 | 9.3 | |
| Platelet count < 150 × 109/L | 1 | INTERMEDIATE 2 | ≥14, <16 | 4.4 | |
| Blasts > 3% | 2 | HIGH | ≥16 | 2 | |
| CALR-unmutated | 2 | / | / | / | |
| Constitutional symptoms | 1 | / | / | / | |
| MIPSS70 | Hemoglobin < 10 g/dL | 1 | LOW | 0–1 | 27.7 |
| WBC > 25 × 109/L | 2 | INTERMEDIATE | 2–4 | 7.1 | |
| Platelet count < 100 × 109/L | 2 | HIGH | ≥5 | 2.3 | |
| Blasts ≥ 2% | 1 | / | / | / | |
| Constitutional symptoms | 1 | / | / | / | |
| BM fibrosis grade ≥ 2 | 1 | / | / | / | |
| Single HMR mutation | 1 | / | / | / | |
| HMR mutations ≥ 2 | 2 | / | / | / | |
| Non-CALR mutation type 1 | 1 | / | / | / | |
|
MIPSS70+
v2.0 |
Severe anemia | 2 | VERY LOW | 0 | NR |
| Moderate anemia | 1 | LOW | 1–2 | 16.4 | |
| VHR karyotype | 4 | INTERMEDIATE | 3–4 | 7.7 | |
| Blasts ≥ 2% | 1 | HIGH | 5–8 | 4.1 | |
| Constitutional symptoms | 2 | VERY HIGH | ≥9 | 1.8 | |
| Unfavorable karyotype | 3 | / | / | / | |
| Single HMR mutation | 2 | / | / | / | |
| HMR mutations ≥ 2 | 3 | / | / | / | |
| Non-CALR mutation type 1 | 2 | / | / | / | |
| GIPSS | Non-CALR mutation type 1 | 1 | LOW | 0 | 26.4 |
| VHR karyotype | 2 | INTERMEDIATE 1 | 1 | 8 | |
| Unfavorable karyotype | 1 | INTERMEDIATE 2 | 2 | 4.2 | |
| ASXL1 mutation | 1 | HIGH | ≥3 | 2 | |
| SRSF2 mutation | 1 | / | / | / | |
| U2AF1 Q157 mutation | 1 | / | / | / | |
| MTSS | HLA-mismatched donor | 2 | LOW | 0–2 | 5 years-OS 83% |
| Non CALR/MPL mutation | 2 | INTERMEDIATE 1 | 3–4 | 5 years-OS 64% | |
| Age > 57 | 1 | INTERMEDIATE 2 | 5 | 5 years-OS 37% | |
| WBC > 25 × 109/L | 1 | HIGH | >5 | 5 years-OS 22% | |
| Platelet count < 150 × 109/L |
1 | / | / | / | |
| Karnofsky score < 90% | 1 | / | / | / | |
| ASXL1 mutation | 1 | / | / | / | |
| RR6 | Spleen length reduction ≤ 30% with respect to baseline at 3 and 6 months |
1.5 | LOW | 0 | NR |
| RBC transfusions requirement at baseline/3 months/6 months |
1 or 1.5 | INTERMEDIATE | 1–2 | 5 | |
| RUX treatment at dose < 20 mg twice daily at baseline, 3, and 6 months |
1 | HIGH | >2 | 2.7 | |
| AIPSS-MF | Sex, age (years), blood blasts (%), hemoglobin (g/L), leukocytes (×109/L), platelet count (×109/L), constitutional symptoms, leukoerythroblastosis |
The model was designed to provide personalized predictions of overall survival and leukemia-free survival. Outcome predictions are from disease diagnosis. |
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