Essential thrombocythemia
ICD-10 D47.3 · ICD-11 3B63.1Z
This protocol addresses the management of essential thrombocythemia (ET) in a specific low-risk population: patients aged 60 years or younger who carry a JAK2 mutation and have no prior thrombotic events.
Clinical Scenario
Patients in this group—aged ≤60 years, JAK2-mutated, and without a thrombosis history—are classified as low-risk ET. Risk stratification in ET integrates age, thrombosis history, and JAK2 mutation status. The presence of a JAK2 mutation in an otherwise low-risk patient defines the clinical subgroup this protocol targets.
Treatment Approach (partial overview)
For low-risk JAK2-mutated ET, antiplatelet therapy with low-dose aspirin plays a central role in management. The structured protocol specifies how this approach is tailored to individual patient characteristics. The complete decision algorithm is available via the link below.
Clinical Goal
The primary aim of treatment in this setting is the alleviation of vasomotor (microvascular) disturbances associated with ET.
DOI: 10.1002/ajh.27216
Figure 5 outlines our general treatment approach in ET, which starts with thrombosis risk stratification: very low (age ≤60 years, no thrombosis history, JAK2 wild-type), low (same as very low but JAK2 mutation present), intermediate (age >60 years, no thrombosis history, JAK2 wild-type), and high (thrombosis history present or age >60 years with JAK2 mutation).
Once- or twice-daily aspirin is strongly advised in low-risk patients, in the absence or presence of CV risk factors, respectively (Figure 5).
On the contrary, we recommend the use of low-dose aspirin (81 mg/day; range 40–100 mg/day) in all patients with low-risk JAK2-mutated ET, provided there are no major contraindications; the latter include clinically significant VWF:RCo <20% associated with bleeding complications.
Low-dose aspirin therapy has also been shown to be effective in alleviating vasomotor (microvascular) disturbances associated with ET or PV.
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