Table 6.
Clinical-Molecular Prognostic Scores in Myelofibrosis
| Prognostic Score | Variables (Points) |
Risk Categories (Points) |
Median Survival (Years) |
|---|---|---|---|
| MIPSS70 Guglielmelli et al67 |
Hemoglobin < 10 g/dL (1) Blasts > 2% (1) Constitutional symptoms (1) Leukocytes > 25 x 109/L (2) Platelet count < 100 x 109/L (2) BM fibrosis ≥ 2 (1) Non CALR type-1 (1) HMRa = 1 (1) HMRa ≥ 2 (2) |
Low (0–1) Intermediate (2–4) High (5–12) |
27.7 7.1 2.3 |
| MIPSS70 plus Guglielmelli et al67 |
Hemoglobin < 10 g/dL (1) Blasts > 2% (1) Constitutional symptoms (1) Non CALR type-1 (2) HMRa = 1 (1) HMRa ≥ 2 (2) Unfavourable karyotypeb (3) |
Low (0–2) Intermediate (3) High (4–6) Very high (7–11) |
20.0 6.3 3.9 1.7 |
| MIPSS70 plus v2.0 Tefferi et al68 |
Hemoglobin 8–10 g/dL (1) Hemoglobin <8 g/dL (2) Blasts > 2% (1) Constitutional symptoms (2) Non CALR type-1 (2) HMRa+U2AF1 Q157 = 1 (2) HMRa+U2AF1 Q157 ≥ 2 (3) HR Karyotypec (3) VHR Karyotyped (4) |
Very low (0) Low (1–2) Intermediate (3–4) High (5–8) Very high (9–14) |
Not reached 10.3 7 3.5 1.8 |
| GIPSS Tefferi et al72 |
Non CALR type-1 (1) ASXL1 mutation (1) SRSF2mutation (1) U2AF1Q157 (1)HR karyotypec (1) VHR karyotyped (2) |
Low (0) Intermediate-1 (1) Intermediate-2 (2) High (3–6) |
26.4 8.0 4.2 2.0 |
| MYSEC-PM Passamonti et al74 |
Hemoglobin < 11 g/dL Blasts ≥ 3% Platelets < 150 x 109/L Constitutional symptoms (2) Age at secondary MF (0.15 point/year) CALR unmutated genotype (2) |
Low (<11) Intermediate-1 (11- <14) Intermediate-2 (14- <16) High (≥ 16) |
Not reached 9.3 4.4 2.0 |
| MTSS Gagelmann et al75 |
Platelets < 150 x 109/L (1) Leukocytes > 25 x 109/L (1) Karnofsky PS < 90% (1) Age ≥ 57 years (1) HLA-mismatched unrelated donor (2) Non CALR/MPL mutation (2) ASXL1mutation (1) |
Low (0–2) Intermediate (3–4) High (5) Very high (6–9) |
5-years OS 83% 5-years OS 64% 5-years OS 37% 5-years OS 22% |
Notes: aHigh molecular risk (HMR) include ASXL1, SRSF2, EZH2, IDH1/2. bUnfavourable karyotype defined any abnormal karyotype other than normal karyotype or sole abnormalities of 20q2, 13q2, +9, chromosome 1 translocation/duplication, -Y, or sex chromosome abnormality other than -Y. cHigh risk (HR) karyotype include all the abnormalities that are not VHR and favourable (normal karyotype or sole abnormalities of 20q−, 13q−, +9, chromosome 1 translocation/duplication or sex chromosome abnormality including -Y). dVery high risk (VHR) include single or multiple abnormalities of −7, inv (3), i(17q), 12p−, 11q−, and autosomal trisomies other than +8 or +9. In bold molecular variables.
Abbreviations: MIPSS, Mutation-Enhanced International Prognostic Scoring System; GIPSS, Genetically Inspired Prognostic Scoring System; MYSEC-PM, Myelofibrosis Secondary to PV and ET-Prognostic Model; MTSS, Myelofibrosis Transplant Scoring System; OS, overall survival.