Table 4.

Prognostic models for patients with ET

Prognostic modelRisk groups and clinical relevance
Conventional score for prediction of vascular complications (European LeukemiaNet recommendations)70  
 At least 1 of the following risk factors:  
  • Age ≥60 y Low risk: age <60 y AND no history of thrombosis or major bleeding AND PLT count <1500 × 109/L, that is, none of the 3 major risk factors 
  • Previous thrombosis or major bleeding High risk: age ≥60 y AND/OR history of thrombosis or major bleeding AND/OR PLT count ≥1500 × 109/L, that is, at least 1 of the 3 major risk factors 
  • PLT count ≥1500 × 109/L While low-risk patients are just followed (observation alone) or given low-dose aspirin, high-risk patients are given a cytoreductive treatment plus low-dose aspirin 
IPSET-thrombosis (International Prognostic Score for ET: estimates the risk of thrombosis)71  
 Risk factors (weight): Low risk: 0-1 point (probability of thrombotic events: 1.03% of patients/year) 
  • Age ≥60 y (1 point) Intermediate risk: 2 points (2.35% of patients/year) 
  • Previous thrombosis (2 points) High risk: ≥3 points (3.56% of patients/year) 
  • Cardiovascular risk factors* (1 point) Potential therapeutic implications: (1) observation alone may be adequate in patients with no risk factors; (2) low-dose aspirin should be used in all patients with JAK2 (V617F) and/or cardiovascular risk factors; (3) older patients (≥60 y) without additional risk factors may not need a cytoreductive treatment; (4) conversely, a cytoreductive treatment may be considered in younger patients (<60 y) with JAK2-mutant ET and concomitant cardiovascular risk factors, even in the absence of previous thrombosis 
  • JAK2 (V617F) mutation (2 points) 
 
IPSET (International Prognostic Score for ET: predicts survival)73  
 Risk factors (weight): Low risk: 0 (median survival not reached) 
  • Age ≥60 y (2 points) Intermediate risk: 1-2 points (median survival, 24.5 y) 
  • Previous thrombosis (1 point) High risk: 3-4 points (median survival, 13.8 y) 
  • Leukocyte count >11 × 109/L (1 point)  
Prognostic modelRisk groups and clinical relevance
Conventional score for prediction of vascular complications (European LeukemiaNet recommendations)70  
 At least 1 of the following risk factors:  
  • Age ≥60 y Low risk: age <60 y AND no history of thrombosis or major bleeding AND PLT count <1500 × 109/L, that is, none of the 3 major risk factors 
  • Previous thrombosis or major bleeding High risk: age ≥60 y AND/OR history of thrombosis or major bleeding AND/OR PLT count ≥1500 × 109/L, that is, at least 1 of the 3 major risk factors 
  • PLT count ≥1500 × 109/L While low-risk patients are just followed (observation alone) or given low-dose aspirin, high-risk patients are given a cytoreductive treatment plus low-dose aspirin 
IPSET-thrombosis (International Prognostic Score for ET: estimates the risk of thrombosis)71  
 Risk factors (weight): Low risk: 0-1 point (probability of thrombotic events: 1.03% of patients/year) 
  • Age ≥60 y (1 point) Intermediate risk: 2 points (2.35% of patients/year) 
  • Previous thrombosis (2 points) High risk: ≥3 points (3.56% of patients/year) 
  • Cardiovascular risk factors* (1 point) Potential therapeutic implications: (1) observation alone may be adequate in patients with no risk factors; (2) low-dose aspirin should be used in all patients with JAK2 (V617F) and/or cardiovascular risk factors; (3) older patients (≥60 y) without additional risk factors may not need a cytoreductive treatment; (4) conversely, a cytoreductive treatment may be considered in younger patients (<60 y) with JAK2-mutant ET and concomitant cardiovascular risk factors, even in the absence of previous thrombosis 
  • JAK2 (V617F) mutation (2 points) 
 
IPSET (International Prognostic Score for ET: predicts survival)73  
 Risk factors (weight): Low risk: 0 (median survival not reached) 
  • Age ≥60 y (2 points) Intermediate risk: 1-2 points (median survival, 24.5 y) 
  • Previous thrombosis (1 point) High risk: 3-4 points (median survival, 13.8 y) 
  • Leukocyte count >11 × 109/L (1 point)  
*

Cardiovascular risk factors include hypertension, diabetes, and active tobacco use.

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