According to a study in the the AJMC
Despite excellent prognoses in low-risk patients with essential thrombocytopenia (ET) and polycythemia vera (PV), knowledge gaps remain and novel, more tolerable therapies require reevaluation of treatment algorithms.
ET and PV are the most common MPNs, which are clonal stem cell neoplasms characterized by proliferation of mature myeloid lineages. The risk of arterial and venous thrombosis, which occurs in 20% to 30% of patients with ET or PV, is the most notable cause of morbidity and mortality in these diseases. Symptom management is the main goal in MPNs, and there are no treatments that alter the disease course as of yet. Even so, the prognosis for patients with low-risk ET and PV is typically measured in decades.
Risk categories for ET and PV based on the International Prognostic Score of Thrombosis for ET (R-UPSET) are as follows:
- Very low-risk (60 years or younger, no history of thrombosis, no JAK2 V617F mutation)
- Low-risk (60 years or younger, no history of thrombosis, presence of JAK2 V617F mutation)
- Intermediate-risk (older than 60 years, no history of thrombosis, no JAK2 V617F mutation)
- High-risk (older than 60 years, presence of JAK2 V617F mutation, or any history of thrombosis)