Prognostic risk stratification scoring systems for MF
| Risk score . | IPSS . | DIPSS . | DIPSS+ . | MYSEC-PM . | MIPSS70 . | MIPSS70+ Version 2.0 . | GIPSS . | |
|---|---|---|---|---|---|---|---|---|
| Applicability . | At diagnosis . | At any time . | At any time . | sMF at diagnosis . | At any time . | At any time . | At any time . | |
| Clinical | Age (y) | >65 [1] | >65 [1] | >65 | Age (0.15/year) | - | - | - |
| Leukocytosis ( × 109/L) | >25 [1] | >25 [1] | >25 | - | >25 [2] | - | - | |
| Blasts | ≥1% [1] | ≥1% [1] | ≥1% | ≥3% [2] | ≥2% [1] | ≥2% [1] | - | |
| Hemoglobin (g/dL) | <10 [1] | <10 [2] | <10 | <11 [2] | <10 [1] | <8 (F)/<9 (M) [2] 8-9.9 (F)/9-10.9 (M) [1] | ||
| Transfusion dependent-anemia | - | - | Yes [1] | - | - | - | - | |
| Platelets ( × 109/L) | - | - | <100 [1] | <150 [1] | <100 [2] | - | - | |
| Constitutional symptoms | Yes [1] | Yes [1] | Yes | Yes [1] | Yes [1] | Yes [2] | ||
| Bone marrow fibrosis | - | - | - | - | Grade ≥2 [1] | - | - | |
| Genetic | Karyotype | - | - | Unfavorable [1] | - | - | Very high risk [4] Unfavorable [3] | Very high risk [2] Unfavorable [1] |
| Driver mutation | - | - | - | Absence of CALR [2] | Absence of type 1 CALR [1] | Absence of type 1 CALR [2] | Absence of type 1 CALR [1] | |
| High-risk molecular mutations | - | - | - | - | HMR mutation [1] >1 HMR [2] | HMR mutation [2] >1 HMR [3] | ASXL1 [1] SRSF2 [1] U2AF1Q157 [1] | |
| Risk category & median OS | Int-2 [2]: 4 y High [3-4]: 2.3 y | Int-2 [3-4]: 4 y High [5-6]: 1.5 y | Int-2 [2-3]: 2.9 y High [4-6]: 1.3 y | Int-2 [15]: 4.4 y High [≥16]: 2 y | Int [2-4]: 7.1 y High [>4]: 2.3 y | Int [3-4]: 7.7 y High [5-8]: 4.1 y Very high [≥9]: 1.8 y | Int-2 [2]: 4.2 y High [≥3]: 2 y | |
| Risk score . | IPSS . | DIPSS . | DIPSS+ . | MYSEC-PM . | MIPSS70 . | MIPSS70+ Version 2.0 . | GIPSS . | |
|---|---|---|---|---|---|---|---|---|
| Applicability . | At diagnosis . | At any time . | At any time . | sMF at diagnosis . | At any time . | At any time . | At any time . | |
| Clinical | Age (y) | >65 [1] | >65 [1] | >65 | Age (0.15/year) | - | - | - |
| Leukocytosis ( × 109/L) | >25 [1] | >25 [1] | >25 | - | >25 [2] | - | - | |
| Blasts | ≥1% [1] | ≥1% [1] | ≥1% | ≥3% [2] | ≥2% [1] | ≥2% [1] | - | |
| Hemoglobin (g/dL) | <10 [1] | <10 [2] | <10 | <11 [2] | <10 [1] | <8 (F)/<9 (M) [2] 8-9.9 (F)/9-10.9 (M) [1] | ||
| Transfusion dependent-anemia | - | - | Yes [1] | - | - | - | - | |
| Platelets ( × 109/L) | - | - | <100 [1] | <150 [1] | <100 [2] | - | - | |
| Constitutional symptoms | Yes [1] | Yes [1] | Yes | Yes [1] | Yes [1] | Yes [2] | ||
| Bone marrow fibrosis | - | - | - | - | Grade ≥2 [1] | - | - | |
| Genetic | Karyotype | - | - | Unfavorable [1] | - | - | Very high risk [4] Unfavorable [3] | Very high risk [2] Unfavorable [1] |
| Driver mutation | - | - | - | Absence of CALR [2] | Absence of type 1 CALR [1] | Absence of type 1 CALR [2] | Absence of type 1 CALR [1] | |
| High-risk molecular mutations | - | - | - | - | HMR mutation [1] >1 HMR [2] | HMR mutation [2] >1 HMR [3] | ASXL1 [1] SRSF2 [1] U2AF1Q157 [1] | |
| Risk category & median OS | Int-2 [2]: 4 y High [3-4]: 2.3 y | Int-2 [3-4]: 4 y High [5-6]: 1.5 y | Int-2 [2-3]: 2.9 y High [4-6]: 1.3 y | Int-2 [15]: 4.4 y High [≥16]: 2 y | Int [2-4]: 7.1 y High [>4]: 2.3 y | Int [3-4]: 7.7 y High [5-8]: 4.1 y Very high [≥9]: 1.8 y | Int-2 [2]: 4.2 y High [≥3]: 2 y | |
DIPSS+ DIPSS Int-1 [1 point], DIPSS Int-2 [2 points] and DIPSS Int-3 [3 points] unfavorable karyotype: complex karyotype or single or 2 abnormalities, including +8, -7/7q-, i(17q), -5/5q-, 12p-, inv(3), or 11q23 rearrangement.
MIPSS 70 HMR mutations: ASXL1, SRSF2, EZH2, IDH1, IDH2. MIPSS70+ Version 2.0 HMR mutations: ASXL1, SRSF2, EZH2, IDH1, IDH2, and U2AF1Q157.
MIPSS70+ Version 2.0 and GIPSS very unfavorable karyotype: single/multiple abnormalities of -7, i(17q), inv(3)/3q21, 12p-/12p11.2, 11q-/11q23, or other autosomal trisomies, not including +8/+9 (eg, +21, +19; Favorable: normal karyotype or sole abnormalities of 13q-, +9, 20q-, chromosome 1 translocation/duplication or sex chromosome abnormality, including-Y; Unfavorable: all other abnormalities.
F, female; HMR, high molecular risk; Int, intermediate; M, male; OS, overall survival; sMF, secondary myelofibrosis; [X] denotes score; y, years.