Disease- and treatment-specific scoring systems
| Factor/value (score) . | Disease specific . | Treatment specific . | |||||
|---|---|---|---|---|---|---|---|
| PMF . | SMF . | Transplant . | Ruxolitinib . | ||||
| DIPSS . | DIPSS+ . | MIPSS70 . | MIPSS70+ v. 2 . | MYSEC-PM . | MTSS . | RR6∗ . | |
| Hemoglobin, g/dL | <10 (2) | <10 (2) | <10 (1) | Moderate anemia† (1) Severe (2) | <11 (2) | — | — |
| Leukocytes, × 109/L | >25 (1) | >25 (1) | >25 (2) | — | — | >25 (1) | — |
| Platelets, × 109/L | — | <100 (1) | <100 (2) | — | <150 (1) | <150 (1) | — |
| Circulating blasts, % | ≥1 (1) | ≥1 (1) | ≥2 (1) | ≥2 (2) | ≥3 (1) | — | — |
| Constitutional symptoms | Yes (1) | Yes (1) | Yes (1) | Yes (2) | Yes (1) | — | — |
| Karnofsky status, % | — | — | — | — | — | <90 (1) | — |
| Fibrosis grade | — | — | ≥2 (1) | — | — | — | — |
| Age, y | >65 (1) | >65 (1) | — | — | × 0.15 | ≥57 (1) | — |
| RBC transfusion dependent | — | Yes (1) | — | — | — | — | At baseline, or 3 and/or 6 mo (1) At all time points (1.5) |
| Cytogenetics | — | Unfavorable‡ (1) | — | Unfavorable§ (3) VHR (4) | — | — | — |
| Driver mutation | — | — | Non-CALR type 1 (1) | Non-CALR type 1 (2) | Non-CALR (2) | Non-CALR/MPL (2) | — |
| Other mutations | — | — | HMR present‖ (1) ≥2 HMR mutations (2) | HMR present¶ (2) ≥2 HMR mutations (3) | — | ASXL1 (1) | — |
| Donor relation | — | — | — | — | — | Mismatched unrelated (2) | — |
| Ruxolitinib dose | — | — | — | — | — | — | <20 mg twice daily at all time points (1) |
| Spleen size | — | — | — | — | — | — | Palpable length reduction ≤30% to baseline at 3 and 6 mo (1.5) |
| Score | Low (0) Intermediate-1 (1-2) Intermediate-2 (3-4) High (≥5) | Low (0) Intermediate-1 (1) Intermediate-2 (2-3) High (4-6) | Low (0-1) Intermediate (2-4) High (≥5) | Very low (0) Low (1-2) Intermediate (3-4) High (5-8) Very high (≥9) | Low (<11) Intermediate-1 (11-13) Intermediate-2 (14-15) High (≥16) | Low (0-2) Intermediate (3-4) High (5) Very high (6-9) | Low (0) Intermediate (1-2) High (≥2.5) |
| Survival (median, 5 y, %) | Low (NR, 95), Intermediate-1 (14 y, 80) Intermediate-2 (4 y, 40), High (2 y, 5) | Low (15 y, 90), Intermediate-1 (7 y, 70) Intermediate-2 (3 y, 40) High (1 y, 10) | Low (NR, 96) Intermediate (6 y, 67) High (3 y, 34) | Very low (NR, 95) Low (1 y, 85) Intermediate (8 y, 70) High (1 y, 40) Very high (2 y, 10) | Low (NR, 90) Intermediate-1 (9 y, 80) Intermediate-2 (4 y, 45) High (2 y, 20) | Low (NR, 90) Intermediate (14 y, 77) High (5 y, 50) Very high (1 y, 34) | Low (NR, 80) Intermediate (5 y, 50) High (3 y, 20) |
| Factor/value (score) . | Disease specific . | Treatment specific . | |||||
|---|---|---|---|---|---|---|---|
| PMF . | SMF . | Transplant . | Ruxolitinib . | ||||
| DIPSS . | DIPSS+ . | MIPSS70 . | MIPSS70+ v. 2 . | MYSEC-PM . | MTSS . | RR6∗ . | |
| Hemoglobin, g/dL | <10 (2) | <10 (2) | <10 (1) | Moderate anemia† (1) Severe (2) | <11 (2) | — | — |
| Leukocytes, × 109/L | >25 (1) | >25 (1) | >25 (2) | — | — | >25 (1) | — |
| Platelets, × 109/L | — | <100 (1) | <100 (2) | — | <150 (1) | <150 (1) | — |
| Circulating blasts, % | ≥1 (1) | ≥1 (1) | ≥2 (1) | ≥2 (2) | ≥3 (1) | — | — |
| Constitutional symptoms | Yes (1) | Yes (1) | Yes (1) | Yes (2) | Yes (1) | — | — |
| Karnofsky status, % | — | — | — | — | — | <90 (1) | — |
| Fibrosis grade | — | — | ≥2 (1) | — | — | — | — |
| Age, y | >65 (1) | >65 (1) | — | — | × 0.15 | ≥57 (1) | — |
| RBC transfusion dependent | — | Yes (1) | — | — | — | — | At baseline, or 3 and/or 6 mo (1) At all time points (1.5) |
| Cytogenetics | — | Unfavorable‡ (1) | — | Unfavorable§ (3) VHR (4) | — | — | — |
| Driver mutation | — | — | Non-CALR type 1 (1) | Non-CALR type 1 (2) | Non-CALR (2) | Non-CALR/MPL (2) | — |
| Other mutations | — | — | HMR present‖ (1) ≥2 HMR mutations (2) | HMR present¶ (2) ≥2 HMR mutations (3) | — | ASXL1 (1) | — |
| Donor relation | — | — | — | — | — | Mismatched unrelated (2) | — |
| Ruxolitinib dose | — | — | — | — | — | — | <20 mg twice daily at all time points (1) |
| Spleen size | — | — | — | — | — | — | Palpable length reduction ≤30% to baseline at 3 and 6 mo (1.5) |
| Score | Low (0) Intermediate-1 (1-2) Intermediate-2 (3-4) High (≥5) | Low (0) Intermediate-1 (1) Intermediate-2 (2-3) High (4-6) | Low (0-1) Intermediate (2-4) High (≥5) | Very low (0) Low (1-2) Intermediate (3-4) High (5-8) Very high (≥9) | Low (<11) Intermediate-1 (11-13) Intermediate-2 (14-15) High (≥16) | Low (0-2) Intermediate (3-4) High (5) Very high (6-9) | Low (0) Intermediate (1-2) High (≥2.5) |
| Survival (median, 5 y, %) | Low (NR, 95), Intermediate-1 (14 y, 80) Intermediate-2 (4 y, 40), High (2 y, 5) | Low (15 y, 90), Intermediate-1 (7 y, 70) Intermediate-2 (3 y, 40) High (1 y, 10) | Low (NR, 96) Intermediate (6 y, 67) High (3 y, 34) | Very low (NR, 95) Low (1 y, 85) Intermediate (8 y, 70) High (1 y, 40) Very high (2 y, 10) | Low (NR, 90) Intermediate-1 (9 y, 80) Intermediate-2 (4 y, 45) High (2 y, 20) | Low (NR, 90) Intermediate (14 y, 77) High (5 y, 50) Very high (1 y, 34) | Low (NR, 80) Intermediate (5 y, 50) High (3 y, 20) |
HMR, high molecular risk; MIPSS70+ v. 2, MIPSS70-plus version 2; NR, not reached; RBC, red blood cell; RR6, ruxolitinib response; SMF, secondary MF (post-PV and –ET MF); VHR, very high risk.
Model to predict survival according to response to ruxolitinib after 6 months.
Moderate (<8 g/dL in women and <9 g/dL in men) and severe (8-9.9 g/dL in women and 9-10.9 g/dL in men).
Complex karyotype: sole or 2 abnormalities, including +8, −7/7q-, i(17q), −5/5q-, 12p-, inv(3) or 11q23 rearrangement.
Unfavorable (chromosomal abnormalities except VHR or sole 13q-, +9, 20q-, chromosome 1 translocation/duplication or sex chromosome alterations including -Y), VHR (single/multiple abnormalities of −7, i(17q), inv(3)/3q21, 12p-/12p11.2, 11q-/11q23, +21, or other autosomal trisomies except +8/9).
HMR (presence and number of ASXL1, EZH2, SRSF2, or IDH1/2).
Original HMR and U2AF1Q157.