Comparison of the performance of prognostic systems in either primary or post-ET/PV myelofibrosis for 5-year survival
| System . | Components . | No. . | C-index (95% CI) . | Bootstrap C-index (95% CI) . |
|---|---|---|---|---|
| Primary myelofibrosis | ||||
| DIPSS | Hemoglobin <10 g/dL, leukocytes >25 × 109/L, circulating blasts ≥1%, age >65 y, constitutional symptoms | 260 | 0.573 (0.664-0.582) | 0.566 (0.557-0.575) |
| DIPSS-plus | DIPSS, transfusion dependence, unfavorable karyotype, platelets <100 × 109/L | 149 | 0.557 (0.546-0.568) | 0.542 (0.531-0.553) |
| MIPSS70 | hemoglobin <10 g/dL, leukocytes >25 × 109/L, platelets <100 × 109/L, circulating blasts ≥2%, fibrosis grade ≥2, constitutional symptoms, absence of CALR type 1-like mutation, HMR category,* ≥2 HMR mutations | 260 | 0.587 (0.578-0.596) | 0.581 (0.572-0.590) |
| MIPSS70-plus version 2.0 | Severity of anemia, circulating blasts ≥2%, constitutional symptoms, absence of CALR type 1-like mutation, HMR category (+U2AF1), ≥2 HMR mutations, 3-tiered cytogenetic risk | 149 | 0.566 (0.558-0.574) | 0.560 (0.551-0.569) |
| GIPSS | Absence of CALR type 1-like mutation; presence of ASXL1, SRSF2, or U2AF1; 3-tiered cytogenetic risk | 149 | 0.544 (0.532-0.556) | 0.532 (0.521-0.543) |
| MTSS | Platelets <150 × 109/L, leukocytes >25 × 109/L, KPS <90%, age ≥57 y, HLA-mismatched unrelated donor, non-CALR/MPL driver mutation genotype, ASXL1 mutation | 260 | 0.718 (0.710-0.726) | 0.710 (0.701-0.719) |
| Post-ET/PV myelofibrosis | ||||
| MYSEC-PM | Hemoglobin <11 g/dL, platelets <150 × 109/L, circulating blasts ≥3%, age, constitutional symptoms, CALR-unmutated genotype | 101 | 0.605 (0.593-0.617) | 0.594 (0.582-0.606) |
| MTSS | 101 | 0.701 (0.690-0.711) | 0.690 (0.679-0.701) |
| System . | Components . | No. . | C-index (95% CI) . | Bootstrap C-index (95% CI) . |
|---|---|---|---|---|
| Primary myelofibrosis | ||||
| DIPSS | Hemoglobin <10 g/dL, leukocytes >25 × 109/L, circulating blasts ≥1%, age >65 y, constitutional symptoms | 260 | 0.573 (0.664-0.582) | 0.566 (0.557-0.575) |
| DIPSS-plus | DIPSS, transfusion dependence, unfavorable karyotype, platelets <100 × 109/L | 149 | 0.557 (0.546-0.568) | 0.542 (0.531-0.553) |
| MIPSS70 | hemoglobin <10 g/dL, leukocytes >25 × 109/L, platelets <100 × 109/L, circulating blasts ≥2%, fibrosis grade ≥2, constitutional symptoms, absence of CALR type 1-like mutation, HMR category,* ≥2 HMR mutations | 260 | 0.587 (0.578-0.596) | 0.581 (0.572-0.590) |
| MIPSS70-plus version 2.0 | Severity of anemia, circulating blasts ≥2%, constitutional symptoms, absence of CALR type 1-like mutation, HMR category (+U2AF1), ≥2 HMR mutations, 3-tiered cytogenetic risk | 149 | 0.566 (0.558-0.574) | 0.560 (0.551-0.569) |
| GIPSS | Absence of CALR type 1-like mutation; presence of ASXL1, SRSF2, or U2AF1; 3-tiered cytogenetic risk | 149 | 0.544 (0.532-0.556) | 0.532 (0.521-0.543) |
| MTSS | Platelets <150 × 109/L, leukocytes >25 × 109/L, KPS <90%, age ≥57 y, HLA-mismatched unrelated donor, non-CALR/MPL driver mutation genotype, ASXL1 mutation | 260 | 0.718 (0.710-0.726) | 0.710 (0.701-0.719) |
| Post-ET/PV myelofibrosis | ||||
| MYSEC-PM | Hemoglobin <11 g/dL, platelets <150 × 109/L, circulating blasts ≥3%, age, constitutional symptoms, CALR-unmutated genotype | 101 | 0.605 (0.593-0.617) | 0.594 (0.582-0.606) |
| MTSS | 101 | 0.701 (0.690-0.711) | 0.690 (0.679-0.701) |
HMR, high-molecular-risk category; MIPSS70, mutation-enhanced International Prognostic Score System for transplantation-age.
High molecular risk category defined as positive for 1 of the mutations: ASXL1, EHZ2, SRSF2, or IDH1/2.