Management of Myeloproliferative Neoplasms

Introduction to Myeloproliferative Neoplasms

Myeloproliferative neoplasms (MPNs) are characterized by a high risk of thrombotic complications, with a pooled prevalence of thrombosis of 28.6% in polycythemia vera (PV) and 20.7% in essential thrombocythemia (ET) 1
Thrombotic events can occur even before diagnosis in patients with MPNs 1

Aspirin significantly reduces thrombotic complications in MPNs, with the ECLAP trial demonstrating a reduced risk of cardiovascular events and major thrombosis (RR 0.40; 95% CI, 0.18–0.91) 1, 2
The National Comprehensive Cancer Network recommends aspirin (81-100 mg/day) plus phlebotomy for low-risk PV, and aspirin alongside cytoreductive therapy for high-risk PV and ET 2
Aspirin (81-100 mg/day) is recommended for patients with low-risk JAK2-mutated ET and those with cardiovascular risk factors 2

Aspirin should be discontinued one week prior to elective surgery and restarted 24 hours after surgery or when bleeding risk is acceptable 2
Regular monitoring, including platelet count (every 1-2 weeks initially) and assessment for thrombotic complications and acquired von Willebrand disease, is recommended 2

Regular monitoring for signs of bleeding and disease progression is necessary, with assessment every 3-6 months 2
Patients with persistent thrombocytopenia (platelet count <100,000/μL) should generally continue low-dose aspirin therapy unless there are signs of active bleeding or the platelet count falls below 10,000/μL, considering the risk of thrombotic events and bleeding complications 2, 3, 4
The American College of Cardiology and the International Society on Thrombosis and Haemostasis (ISTH) provide guidelines for managing thrombocytopenia and aspirin therapy, with individual risk assessment and hematology consultation recommended 3, 4
Temporary discontinuation of aspirin and consideration of platelet transfusion may be necessary in cases of severe thrombocytopenia or active bleeding, with daily platelet count monitoring and mandatory hematology consultation 3