Table 2.
Prognostic models to predict survival in PMF and SMF
| Variables (weight) | Score |
|||||
|---|---|---|---|---|---|---|
| DIPSS | DIPSS+ | MIPSS-70 | MIPSS-70+ version 2.0 | MYSEC-PM | MTSS | |
| Clinical features | Age >65 y (1) Constitutional symptoms (1) |
Age >65 y (1) Constitutional symptoms (1) RBC transfusions need (1) |
Constitutional symptoms (1) |
Constitutional symptoms (2) |
Age (0.15 × y of age) Constitutional symptoms (1) |
Age ≥57 y (1) Karnofsky <90% (1) MMUD (2) |
| Complete blood count | Hb <10 g/dL (2) WBC >25 × 109/L (1) Blasts ≥1% (1) |
Hb <10 g/dL (1) WBC >25 × 109/L (1) Blasts ≥1% (1) PLT <100 × 109/L (1) |
Hb <10 g/dL (1) WBC >25 × 109/L (2) Blasts ≥2% (1) PLT <100 × 109/L (2) |
Severe anemia∗ (2) Moderate anemia† (1) Blasts ≥2% (1) |
Hb <11 g/dL (2) Blasts ≥3% (2) PLT <150 × 109/L (1) |
WBC >25 × 109/L (1) PLT <150 × 109/L (1) |
| Driver mutation status | Absence of CALR type 1/like (1) | Absence of CALR type 1/like (2) | Absence of CALR (2) | Absence of CALR/MPL (2) | ||
| Additional myeloid-gene mutations | 1 HMR (1) ≥2 HMR (2) |
1 HMR included U2AF1Q157 (2) ≥2 HMR included U2AF1Q157 (3) |
ASXL1 (1) | |||
| Cytogenetics | Unfavorable‡ (1) | Unfavorable§ (3) Very high-risk‖ (4) |
||||
| BM morphology | BMF grade ≥2 (1) | |||||
|
Risk category (score), median survival¶ |
Low (0), NR Int-1 (1-2), 14.2 y Int-2 (3-4), 4 y High (5-6), 1.5 y |
Low (0), 15.4 y Int-1 (1), 6.5 y Int-2 (2-3), 2.9 y High (≥4), 1.3 y |
Low (0-1), NR Int (2-4), 6.3 y High (≥5), 3.1 y |
Very low (0), NR Low (1-2), 16.4 y Int (3-4), 7.7 y High (5-8), 4.1 y Very high (≥9), 1.8 y |
Low (<11), NR Int-1 (11-13), 9.3 y Int-2 (14-15), 4.4 y High (≥16), 2 y |
Low (0-2), 90% Int (3-4), 77% High (5), 50% Very high (6-9), 34% |
BM, bone marrow; BMF, bone marrow fibrosis; DIPSS, Dynamic International Prognostic Scoring System; Hb, hemoglobin; HMR, high molecular risk (one among ASXL1, EZH2, SRSF2, or IDH1/2); Int, intermediate; MIPSS, Mutation-Enhanced International Prognostic Score System; MMUD, mismatched unrelated donor; MTSS, MF Transplant Scoring System; MYSEC-PM, Secondary to Polycythemia Vera and Essential Thrombocythemia Prognostic Model; NR, not reached; PLT, platelets; RBC, red blood cells; WBC, white blood cells.
Hb <8 g/dL in women and Hb <9 g/dL in men.
Hb 8 to 9.9 g/dL in women and Hb 9 to 10.9 g/dL in men.
Complex karyotype or sole or 2 abnormalities including +8, −7/7q-, i(17q), −5/5q-, 12p-, inv(3) or 11q23 rearrangement.
Chromosomal abnormalities except “very high-risk” (see below) or sole 13q-, +9, 20q-, chromosome 1 translocation/duplication or sex chromosome alterations including -Y.
Single/multiple abnormalities of −7, i(17q), inv(3)/3q21, 12p-/12p11.2, 11q-/11q23, +21, or other autosomal trisomies except +8/9.
Median survival for DIPSS, DIPSS+, MIPSS-70, MIPSS-70+ version 2.0 and MYSEC-PM; 5-year survival for MTSS.