Table 1.
List of the available risk score for MF and their prognostic relevance. PMF: Primary Myelofibrosis; sMF: Secondary Myelofibrosis; HMR: high molecular risk; Int: intermediate; TD transfusion-dependent; BM: bone marrow; OS: overall survival.
| Risk Score | IPSS | DIPSS | DIPSS-Plus | MYSEC-PM | MIPSS70 | MIPSS70-Plus v2.0 | GIPSS | |
|---|---|---|---|---|---|---|---|---|
| Applicability | PMF at diagnosis |
PMF at any time |
PMF at any time |
sMF at diagnosis |
PMF at any time |
PMF at any time |
PMF at any time |
|
| Features | Clinical | Clinical | Clinical & Molecular |
Clinical & molecular |
Clinical & molecular |
Clinical & molecular |
Genetical only | |
| Items (points) | Age | >65 y (1) | >65 y (1) | >65 y (1) | Age (0.15/y) | – | – | – |
| Leucocytes | >25 × 109/L (1) | >25 × 109/L (1) | >25 × 109/L (1) | – | >25 × 109/L (1) | – | – | |
| Blasts | ≥1% (1) | ≥1% (1) | ≥1% (1) | ≥3% (2) | ≥2% (2) | ≥2% (2) | – | |
| Constitutional symptoms | Yes/No (1) | Yes/No (1) | Yes/No (1) | Yes/No (1) | Yes/No (1) | Yes/No (2) | – | |
| Hemoglobin | <10 g/dL (1) | <10 g/dL (2) | <10 g/dL (2) | <11 g/dL (2) | <10 g/dL (1) | <8 g/dL (F)/<9 g/dL (M) (2) 8–9.9 g/dL (F)/9–10.9 g/dL (M) (1) |
– | |
| TD-anemia | – | – | Yes/No (1) | – | – | – | – | |
| Cytogenetics | – | – | Unfavorable £ (1) | – | – | very high risk § (4) unfavorable § (3) |
very high risk § (2) unfavorable § (1) |
|
| Platelets | – | – | <100 × 109/L (1) | <150 × 109/L (2) | <100 × 109/L (2) | – | – | |
| Molecular | – | – | – | No CALR (2) | No CALR type-1 (1) HMR % mutation (1) >1 HMR % mutations (2) |
No CALR type-1 (2) HMR $ mutation (2) >1 HMR $ mutations (3) |
No CALR type-1 (1) ASXL1 (1) SRSF2 (1) U2AF1Q (1) |
|
| BM fibrosis | – | – | – | – | Grade ≥2 (1) | – | – | |
| Higher risk Categories (score) 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 (>14 < 16): 4.4 y High (≥16): 2 y |
Int (2–4): 7.1 y High (>4): 2.3 y |
High (5–8): 4.1 y Very high (≥9): 1.8 y |
Int-2 (2): 4.2 y High (≥3): 2 y |
|
£ Unfavorable karyotype: complex karyotype or single or two abnormalities, including +8, −7/7q-, i(17q), −5/5q-, 12p-, inv(3), or 11q23 rearrangement. % HMR mutations according to MIPSS70: ASXL1, SRSF2, EZH2, IDH1, IDH2. $ HMR mutations according to MIPSS70-plus v2.0: ASXL1, SRSF2, EZH2, IDH1, IDH2, and U2AF1Q157. § 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 (e.g., +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.