Table 4.

Risk adapted treatment of patients with ET

Risk categoryOur therapeutic approach
Low risk (age <60 y AND no history of thrombosis or major bleeding AND PLT count <1500 × 109/L, ie, none of the 3 major risk factors) JAK2 (V617F)-mutant ET 
• Low-dose aspirin* 
CALR-mutant ET, MPL-mutant ET and triple-negative patients 
• Low-dose aspirin* in patients with at least 1 concomitant cardiovascular risk factor and/or with microvascular symptoms 
• Observation alone or low-dose aspirin* in patients without concomitant cardiovascular risk factors and without microvascular symptoms (individual decision-making) 
High risk (age ≥60 y AND/OR history of thrombosis or major bleeding AND/OR PLT count ≥1500 × 109/L, ie, at least 1 of the 3 major risk factors) • Low-dose aspirin* + cytoreductive therapy 
Risk categoryOur therapeutic approach
Low risk (age <60 y AND no history of thrombosis or major bleeding AND PLT count <1500 × 109/L, ie, none of the 3 major risk factors) JAK2 (V617F)-mutant ET 
• Low-dose aspirin* 
CALR-mutant ET, MPL-mutant ET and triple-negative patients 
• Low-dose aspirin* in patients with at least 1 concomitant cardiovascular risk factor and/or with microvascular symptoms 
• Observation alone or low-dose aspirin* in patients without concomitant cardiovascular risk factors and without microvascular symptoms (individual decision-making) 
High risk (age ≥60 y AND/OR history of thrombosis or major bleeding AND/OR PLT count ≥1500 × 109/L, ie, at least 1 of the 3 major risk factors) • Low-dose aspirin* + cytoreductive therapy 

Risk stratification is based on the European LeukemiaNet recommendations: age ≥60 y, history of thrombosis or major bleeding, and PLT count ≥1500 × 109/L are the 3 risk factors used for this stratification.

*

If PLT count is ≥1000 × 109/L and/or clinical manifestations include bleeding, the possibility of an acquired von Willebrand syndrome (AVWS) should be considered: von Willebrand factor antigen level and ristocetin cofactor activity should therefore be assessed. If AVWS is diagnosed, the use of low-dose aspirin is contraindicated; in any case, we do not use aspirin in high-risk patients whose PLT count is ≥1500 × 109/L.

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

We provide the patient with the information he/she needs to understand the benefit-risk balance of taking low-dose aspirin for years, considering age, occupation, and lifestyle, and then support his/her choice.

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