New Clinical Practice Guidelines, November 2017

John Anello; Brian Feinberg; Richard Lindsey; Cristina Wojdylo; Olivia Wong, DO; Yonah Korngold; John Heinegg; Sam Shlomo Spaeth


November 08, 2017

In This Article

Myeloproliferative Neoplasms

National Comprehensive Cancer Network

Advanced age (>60 years) and history of thrombosis are the most consistent risk factors associated with risk of thrombosis.

Leukocytosis at diagnosis is associated with a higher risk of thrombosis and major hemorrhage in patients with polycythemia vera (PV) and essential thrombocythemia (ET).

Age ≥60 years, leukocyte count ≥11 x 109/L, and prior thrombosis are significantly associated with inferior survival.

The safety and efficacy of low-dose aspirin for the prevention of thrombotic complications in PV was established in a multicenter trial of patients with no contraindication to aspirin therapy and no history of a thrombotic event.

Hydroxyurea, interferon alfa, and peginterferon alfa have been shown to be effective for the prevention of thrombotic complications in patients with PV.

Hydroxyurea, interferon alfa, and peginterferon alfa, and possibly anagrelide, have been shown to be effective for the prevention of venous thrombotic complications in patients with high-risk ET.

Treatment options should be individualized based on age and history of thrombosis for patients with PV.

Aspirin (81-100 mg/day) and phlebotomy (to maintain a hematocrit level of <45%) are recommended for all patients with low-risk PV. Cytoreductive therapy is not recommended as initial treatment.

In addition to aspirin and phlebotomy, cytoreductive therapy is also used to reduce the risk of thrombotic complications for patients with high-risk PV. Cytoreductive therapy (hydroxyurea) with aspirin (81–100 mg/day) for vascular symptoms and phlebotomy (to maintain a hematocrit level of <45%) is recommended as initial treatment. Interferon alfa-2b, peginterferon alfa-2a, or peginterferon alfa-2b could be considered for younger patients, pregnant patients requiring cytoreductive therapy, or patients requiring cytoreductive therapy who defer hydroxyurea.

Monitoring for new thrombosis, acquired von Willebrand disease (VWD), and/or disease-related major bleeding (in patients with ET) and management of cardiovascular risk factors are recommended for all patients. After initiation of low-dose aspirin (and phlebotomy for patients with PV), it is recommended to monitor symptom status using the MPN Symptom Assessment Form Total Symptom Score (MPN-SAF TSS), to evaluate for signs and symptoms of disease progression every 3 to 6 months, and to assess for potential indications for cytoreductive therapy. Bone marrow aspirate and biopsy should be performed as clinically indicated (if supported by increased symptoms and signs of progression).

The thrombotic and bleeding risk should be strongly considered before elective surgery because patients with PV and ET are at higher risk for bleeding despite optimal management.

Pregnancy is considered a high-risk clinical situation in patients with PV and ET. The presence of JAK2 V617F mutation is an adverse prognostic factor for pregnancy outcome, and pregnancy complications are associated with a higher risk of subsequent thrombotic events in patients with ET. Use of aspirin has been reported to be effective in reducing pregnancy complications, especially in patients with JAK2-mutated ET.

Evaluation by a high-risk obstetrician should be considered before conception, and consultation with a high-risk obstetrician and an obstetric anesthesiologist is recommended regarding the optimal timing for discontinuation of anticoagulant therapy in preparation for an epidural before delivery.


  • Mesa RA, Jamieson C, Bhatia R, et al. NCCN guidelines insights: myeloproliferative neoplasms, version 2.2018. J Natl Compr Canc Netw. 2017 Oct;15(10):1193-207.


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