Figure 2.

Proposed treatment stratification for pediatric ET showing details of our group’s proposed treatment stratification for children with ET. Asymptomatic patients are observed with monitoring of blood counts. Patients with lower-risk symptoms such as organomegaly, erythromelalgia, or headache, or those with additional cardiovascular or thrombophilia risk factors (such as elevated cholesterol or Factor V Leiden mutation) are treated with aspirin and are monitored for change in symptoms. High-risk patients, including those with thrombosis or severe bleeding, those who failed aspirin therapy, or those who have persistent extreme thrombocytosis, are treated with cytoreductive therapy. Our current first line is hydroxyurea, followed by Interferon-alpha or anagrelide in certain patients. Ruxolitinib is not currently first-line therapy but is being studied in clinical trial for children with MPN and targeted inhibitors may some day become an important component of therapy for children with MPN.