Initial Treatment Recommendations for Myeloproliferative Diseases (MPD)

Risk Stratification

The National Comprehensive Cancer Network recommends that high-risk patients with myeloproliferative diseases, including those with age ≥60 years, history of prior thrombosis, receive cytoreductive therapy in addition to aspirin and phlebotomy 1, 2
The National Comprehensive Cancer Network recommends that low-risk patients with myeloproliferative diseases, including those with age <60 years, no history of thrombosis, receive aspirin and phlebotomy 1

The National Comprehensive Cancer Network recommends phlebotomy to maintain hematocrit <45% for low-risk PV patients 1, 2
The National Comprehensive Cancer Network recommends low-dose aspirin (81-100 mg/day) for low-risk PV patients 1, 2
The National Comprehensive Cancer Network recommends cytoreductive therapy with hydroxyurea as first-line option for high-risk PV patients 1, 2

The National Comprehensive Cancer Network recommends observation for very low-risk or low-risk ET patients 1
The National Comprehensive Cancer Network recommends low-dose aspirin (81-100 mg/day) for high-risk ET patients 1
The National Comprehensive Cancer Network recommends cytoreductive therapy with hydroxyurea as first-line option for high-risk ET patients 1, 3

The National Comprehensive Cancer Network recommends hydroxyurea as first-line cytoreductive agent for high-risk PV and ET 1, 2, 3
The National Comprehensive Cancer Network recommends interferon alpha for younger patients or pregnant patients requiring cytoreductive therapy 1, 2

The National Comprehensive Cancer Network recommends evaluating patients every 3-6 months for new thrombosis or bleeding, disease-related symptoms, need for frequent phlebotomy, progressive splenomegaly, and progressive leukocytosis 1, 4
The National Comprehensive Cancer Network recommends targeting a platelet count <400 × 10^9/L, WBC count <10 × 10^9/L, and hematocrit <45% (for PV) 3, 4

The National Comprehensive Cancer Network recommends aggressively managing cardiovascular risk factors in all patients to reduce thrombosis risk 2
The National Comprehensive Cancer Network recommends monitoring for acquired von Willebrand disease in patients with extreme thrombocytosis 1