Essential Thrombocytopenia

Workup

If the platelet count is >1000, then acquired vWF syndrome must be ruled out by measurement of ristocetin cofactor activity.

Management of ET

Platelet-Lowering Agent

1. Hydroxyurea (first-line)
2. Anagrelide (alternative)

ASA

ASA is indicated for all but very low-risk disease (for thrombosis). Factors that increase the risk of thrombosis are increased age, JAK2 positive, history of thrombosis.

However, if there is acquired von Willebrand syndrome (as defined by low ristocetin cofactor activity), then hold off on ASA initiation until that is resolved (by cytoreduction).

References

1. NEJM Knowledge+
2. How I treat essential thrombocythemia - PubMed