Molecularly integrated risk scores in patients with PMF and sMF
| . | DIPSS-plus . | MIPSS70 . | MIPSS70/plus v2.0 . | GIPSS . | MYSEC-PM . | MTSS . |
|---|---|---|---|---|---|---|
| . | Prognostic variables (points) . | |||||
| Age, y | >65 (1) | — | — | — | 0.15 point/y | ≥57 (1) |
| Constitutional symptoms | Present (1) | Present (1) | Present (2) | — | Present (1) | — |
| Hemoglobin, g/dL | <10.0 (2) | <10 (1) | 8-9.9 F; 9-10.9 M (1) <8 F, <9 M (2) | — | <11 (2) | — |
| Leukocyte count, × 109/L | >25.0 (1) | >25.0 (2) | — | — | >25.0 (1) | |
| Circulating blast cells, % | ≥1% (1) | ≥2% (1) | ≥2% (1) | — | ≥3% (2) | — |
| Platelet count, × 109/L | <100 (1) | <100 (2) | — | — | <150 (1) | <150 (1) |
| RBC transfusion need | Yes (1) | — | — | — | — | — |
| Karyotype | Unfavorable (1) | — | High risk (3) Very high risk (4) | High risk (1) Very high risk (2) | — | — |
| BM fibrosis grade | — | ≥MF2 (1) | — | — | — | — |
| Absence of CALR | — | Yes (1) | Yes (2) | Yes (1) | Yes (2) | Yes/absence of MPL (2) |
| Molecular profile | — | HMR 1 mut (1) HMR ≥2 mut (2) | HMR+U2AF1 Q157 1 mut (2) HMR+U2AF1 Q157 ≥ 2 mut (3) | ASXL1 (1) SRSF2 (1) U2AF1 Q157 (1) | — | ASXL1 (1) |
| Karnofsky PS | — | — | — | — | — | < 90% (1) |
| HLA donor | — | — | — | — | — | MUD (2) |
| Risk group (score): Overall survival in years | ||||||
| Categories | Low (0):15 y Intermediate 1 (1): 6.7 y Intermediate 2 (2-3): 2.9 y High (≥4): 1.3 y | Low (0-1): 27.7 y Intermediate (2-4): 7.1 y High (≥5): 2.3 y | Very low (0): not reached Low (1-2): 10.3 y Intermediate (3-4): 7.0 y High (5-8): 3.5 y Very high (≥9): 1.8 y | Low (0): 26.4 y Intermediate 1 (1): 8.0 y Intermediate 2 (2): 4.2 y High (3-6): 2.0 y | Low (<11): not reached Intermediate 1 (11-14): 9.3 y Intermediate 2 (14-16): 4.4 y High (≥16): 2.0 y | Low (0-2): 90%* Intermediate (3-4): 77%* High (5): 50%* Very high (6-9): 34%* |
| When/for whom | During follow-up; PMF patients | At diagnosis; PMF patients | At diagnosis; PMF patients | At diagnosis; PMF patients | At diagnosis; sMF | MF patients planned for SCT |
| Website tool | https://qxmd.com/calculate/calculator_315/dipss-plus-score-for-prognosis-in-myelofibrosis | www.mipss70score.it | www.mipss70score.it | NA | www.mysec-pm.eu | NA |
| Molecular profile description | Karyotype description | |||||
| DIPSS-plus | Unfavorable: complex karyotype or single or 2 abnormalities, including +8, −7/7q−, i(17q), −5/5q−, 12p−, inv(3), or 11q23 rearrangement | |||||
| MIPSS70 | Absence of CALR type 1 HRM: mutation in at least 1 of these mutated genes: ASXL1, SRSF2, IDH1, IDH2, EZH2 | Not included | ||||
| MIPSS70/plus v2.0 | Absence of CALR type 1 HMR: mutation in at least 1 of these mutated genes: ASXL1, SRSF2, IDH1, IDH2, EZH2, U2AF1 Q157 | High risk (HR): all the abnormalities that are not VHR and favorable (normal karyotype or sole abnormalities of 20q−, 13q−, +9, chromosome 1 translocation/duplication, or sex chromosome abnormality including −Y) Very high risk (VHR): includes single or multiple abnormalities of −7, inv (3), I (17q), 12p−, 11q−, and autosomal trisomies other than +8 or +9 | ||||
| GIPSS | Mutation in ASXL1, SRSF2, U2AF1 Q157 | Same as MIPPS70/plus v2.0 | ||||
| MYSEC-PM | Absence of CALR (all types) | Not included | ||||
| MTSS | Absence of CALR (all types)/MPL Mutation in ASXL1 | Not included | ||||
| . | DIPSS-plus . | MIPSS70 . | MIPSS70/plus v2.0 . | GIPSS . | MYSEC-PM . | MTSS . |
|---|---|---|---|---|---|---|
| . | Prognostic variables (points) . | |||||
| Age, y | >65 (1) | — | — | — | 0.15 point/y | ≥57 (1) |
| Constitutional symptoms | Present (1) | Present (1) | Present (2) | — | Present (1) | — |
| Hemoglobin, g/dL | <10.0 (2) | <10 (1) | 8-9.9 F; 9-10.9 M (1) <8 F, <9 M (2) | — | <11 (2) | — |
| Leukocyte count, × 109/L | >25.0 (1) | >25.0 (2) | — | — | >25.0 (1) | |
| Circulating blast cells, % | ≥1% (1) | ≥2% (1) | ≥2% (1) | — | ≥3% (2) | — |
| Platelet count, × 109/L | <100 (1) | <100 (2) | — | — | <150 (1) | <150 (1) |
| RBC transfusion need | Yes (1) | — | — | — | — | — |
| Karyotype | Unfavorable (1) | — | High risk (3) Very high risk (4) | High risk (1) Very high risk (2) | — | — |
| BM fibrosis grade | — | ≥MF2 (1) | — | — | — | — |
| Absence of CALR | — | Yes (1) | Yes (2) | Yes (1) | Yes (2) | Yes/absence of MPL (2) |
| Molecular profile | — | HMR 1 mut (1) HMR ≥2 mut (2) | HMR+U2AF1 Q157 1 mut (2) HMR+U2AF1 Q157 ≥ 2 mut (3) | ASXL1 (1) SRSF2 (1) U2AF1 Q157 (1) | — | ASXL1 (1) |
| Karnofsky PS | — | — | — | — | — | < 90% (1) |
| HLA donor | — | — | — | — | — | MUD (2) |
| Risk group (score): Overall survival in years | ||||||
| Categories | Low (0):15 y Intermediate 1 (1): 6.7 y Intermediate 2 (2-3): 2.9 y High (≥4): 1.3 y | Low (0-1): 27.7 y Intermediate (2-4): 7.1 y High (≥5): 2.3 y | Very low (0): not reached Low (1-2): 10.3 y Intermediate (3-4): 7.0 y High (5-8): 3.5 y Very high (≥9): 1.8 y | Low (0): 26.4 y Intermediate 1 (1): 8.0 y Intermediate 2 (2): 4.2 y High (3-6): 2.0 y | Low (<11): not reached Intermediate 1 (11-14): 9.3 y Intermediate 2 (14-16): 4.4 y High (≥16): 2.0 y | Low (0-2): 90%* Intermediate (3-4): 77%* High (5): 50%* Very high (6-9): 34%* |
| When/for whom | During follow-up; PMF patients | At diagnosis; PMF patients | At diagnosis; PMF patients | At diagnosis; PMF patients | At diagnosis; sMF | MF patients planned for SCT |
| Website tool | https://qxmd.com/calculate/calculator_315/dipss-plus-score-for-prognosis-in-myelofibrosis | www.mipss70score.it | www.mipss70score.it | NA | www.mysec-pm.eu | NA |
| Molecular profile description | Karyotype description | |||||
| DIPSS-plus | Unfavorable: complex karyotype or single or 2 abnormalities, including +8, −7/7q−, i(17q), −5/5q−, 12p−, inv(3), or 11q23 rearrangement | |||||
| MIPSS70 | Absence of CALR type 1 HRM: mutation in at least 1 of these mutated genes: ASXL1, SRSF2, IDH1, IDH2, EZH2 | Not included | ||||
| MIPSS70/plus v2.0 | Absence of CALR type 1 HMR: mutation in at least 1 of these mutated genes: ASXL1, SRSF2, IDH1, IDH2, EZH2, U2AF1 Q157 | High risk (HR): all the abnormalities that are not VHR and favorable (normal karyotype or sole abnormalities of 20q−, 13q−, +9, chromosome 1 translocation/duplication, or sex chromosome abnormality including −Y) Very high risk (VHR): includes single or multiple abnormalities of −7, inv (3), I (17q), 12p−, 11q−, and autosomal trisomies other than +8 or +9 | ||||
| GIPSS | Mutation in ASXL1, SRSF2, U2AF1 Q157 | Same as MIPPS70/plus v2.0 | ||||
| MYSEC-PM | Absence of CALR (all types) | Not included | ||||
| MTSS | Absence of CALR (all types)/MPL Mutation in ASXL1 | Not included | ||||
According to National Comprehensive Cancer Network guidelines, in patients with MF, a lower-risk category includes the following combination: MIPSS70 ≤ 3, MIPSS70v2 ≤ 3, DIPSS-plus ≤1, DIPSS ≤2, and MYSEC-PM <14, while a higher-risk category includes MIPSS70 ≥ 4, MIPSS70v2 ≥ 4, DIPSS-plus >1, DIPSS >2, and MYSEC-PM ≥14.
5-year overall survival (learning-cohort).
HLA, human leukocyte antigen; NA, not available; PS, performance status; RBC, red blood cell.