This protocol applies to patients older than 60 years with essential thrombocythemia, no prior thrombosis, JAK2 wild-type status, and cardiovascular risk factors, in whom first-line therapy has not maintained adequate platelet control.
Intermediate-risk essential thrombocythemia: age >60 years, no history of thrombosis, JAK2 wild-type, with cardiovascular risk factors. Risk stratification places this group in the intermediate category, where age and cardiovascular risk factors together inform treatment selection.
First-line therapy consisted of hydroxyurea combined with once-daily low-dose aspirin. Escalation to this protocol is triggered when that regimen has not achieved the primary goal: maintaining platelet count in the normal range.
Platelet count kept in the normal range.
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).
Twice-daily aspirin is also our current treatment choice for intermediate-risk disease, but combination of a cytoreductive drug with once-daily aspirin is a reasonable alternative in intermediate-risk patients with CV risk factors (Figure 5).
ET patients who are either intolerant or resistant to HU are effectively managed by IFN-α (pegylated preparations preferred) or busulfan.
Among these 2 s-line drugs, we prefer the use of IFN-α for patients younger than age 65 years and busulfan in the older age group, although there is no controlled evidence to support or refute such a strategy.
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