Tratamento da Trombocitemia Essencial em Pacientes com Menos de 60 Anos sem Histórico de Trombose, Status JAK2 Wild-Type e Fatores de Risco Cardiovascular ou Mutação CALR-1/MPL
Este protocolo aborda um subgrupo específico de trombocitemia essencial: pacientes com 60 anos ou menos, sem histórico prévio de trombose e status mutacional JAK2 wild-type, que também apresentam fatores de risco cardiovascular ou albergam uma mutação driver CALR-1 ou MPL. Esta combinação define um nível particular de risco de trombose que orienta a abordagem de manejo.
O risco de trombose no status mutacional driver triplo negativo isolado é considerado baixo demais para requerer tratamento — a presença de fatores de risco cardiovascular ou mutação CALR-1/MPL é o que aciona a abordagem de manejo descrita aqui.
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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