Essential thrombocythemia
ICD-10 D47.3 · ICD-11 3B63.1Z

Treatment of Essential Thrombocythemia Under Age 60 with No Thrombosis History, JAK2 Wild-Type Status, and Cardiovascular Risk Factors or CALR-1/MPL Mutation

This protocol addresses a specific subset of essential thrombocythemia: patients aged 60 years or younger, with no prior history of thrombosis and a JAK2 wild-type mutational status, who also carry cardiovascular risk factors or harbour a CALR-1 or MPL driver mutation. This combination defines a particular thrombosis risk tier that informs the management approach.

Clinical scenario All of the following must be present:
Age ≤60 years No thrombosis history JAK2 wild-type
Cardiovascular risk factors — or  CALR-1 mutation or  MPL mutation

Thrombosis risk in triple-negative driver mutational status alone is considered too low to require treatment — the presence of cardiovascular risk factors or a CALR-1/MPL mutation is what triggers the management approach described here.

First-line approach (partial overview) The evidence-based approach for this risk profile involves low-dose aspirin therapy — the full protocol, including selection criteria and all clinical specifics, is available via the link below.
Treatment goal Alleviation of vasomotor (microvascular) disturbances associated with essential thrombocythemia.
Instant Access to Structured Evidence-Based Regimens

References

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).

Thrombosis risk in very low risk patients with triple-negative driver mutational status is too low to warrant the need for any form of therapy, but once-daily aspirin therapy is advised in the presence of either CV risk factors or CALR-1/MPL mutations.

Very low-risk patients with ET might not require any therapy, unless in the presence of CV risk factors or CALR-1/MPL mutations, where once-daily low-dose aspirin is advised (Figure 5).

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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