Table 1

MPN complication rates, prognosis and risk scoring algorithms

ETPVPMF
Thrombotic events 10%–29%23  34%–39%23  7.2–13.2%65,66  
Bleeding events 0.3%67  2.968  – 
Leukemic transformation 2% at 15 y69,70  5.5% at 15 y27  6%–18%71  
Overall survival 14.7 y70  6.5–24 y72,73  6–10 y28,74,75  
Risk algorithms IPSET26  Tefferi criteria27  DIPSS PLUS 
 Age ≥60 (2 pts) vs <60 ≥67 (5 pts) 57–66 (2 pts) ≥65 (1 pt) vs <65 
 Leukocytes ≥11 (1 pt) vs <11 × 109/L ≥15 (1 pt) vs <15 × 109/L >25 (1 pt) vs ≤25 × 109/L 
 Prior vascular events Yes (1 pt) vs no Yes (1 pt) vs no  
 Anemia   <10 (2 pts) vs ≥10g/dL 
 Constitutional symptoms   Present* (1 pt) vs absent 
 Peripheral blood blasts   ≥1% (1 pt) vs <1% 
 Unfavorable karyotype   Present (1 pt) vs absent 
RBC transfusion requirement   Present (1 pt) vs absent 
Platelet count < 100 000 × 10/L   Present (1 pt) vs absent 
High risk 3–4 points 4 points >4 points 
Intermediate 2 risk N/A 3 points 3–4 points 
Intermediate 1 risk 1–2 points 1–2 points 1–2 points 
Low risk 0 points 0 points 
ETPVPMF
Thrombotic events 10%–29%23  34%–39%23  7.2–13.2%65,66  
Bleeding events 0.3%67  2.968  – 
Leukemic transformation 2% at 15 y69,70  5.5% at 15 y27  6%–18%71  
Overall survival 14.7 y70  6.5–24 y72,73  6–10 y28,74,75  
Risk algorithms IPSET26  Tefferi criteria27  DIPSS PLUS 
 Age ≥60 (2 pts) vs <60 ≥67 (5 pts) 57–66 (2 pts) ≥65 (1 pt) vs <65 
 Leukocytes ≥11 (1 pt) vs <11 × 109/L ≥15 (1 pt) vs <15 × 109/L >25 (1 pt) vs ≤25 × 109/L 
 Prior vascular events Yes (1 pt) vs no Yes (1 pt) vs no  
 Anemia   <10 (2 pts) vs ≥10g/dL 
 Constitutional symptoms   Present* (1 pt) vs absent 
 Peripheral blood blasts   ≥1% (1 pt) vs <1% 
 Unfavorable karyotype   Present (1 pt) vs absent 
RBC transfusion requirement   Present (1 pt) vs absent 
Platelet count < 100 000 × 10/L   Present (1 pt) vs absent 
High risk 3–4 points 4 points >4 points 
Intermediate 2 risk N/A 3 points 3–4 points 
Intermediate 1 risk 1–2 points 1–2 points 1–2 points 
Low risk 0 points 0 points 
*

Constitutional symptoms were defined as weight loss over 6 months, night sweats, unexplained fever.29 

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