NCCN Guidelines Updated for Myeloproliferative Neoplasms

Experts have updated and published the National Comprehensive Cancer Network (NCCN) guidelines for myeloproliferative neoplasms (MPNs).

The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for MPN was published by Aaron T. Gerds, MD, MS, of the Cleveland Clinic, and colleagues in the Journal of the National Comprehensive Cancer Network.

The current guidelines review molecular abnormalities in MPN, including JAK2 V617, CALR, and MPL mutations; the diagnostic classification and work-up of MPN; assessment of symptom burden; and symptom management in patients with MPN.

The guidelines include a section on the management of myelofibrosis (MF), which includes risk stratification, treatment options, treatment recommendations based on symptom assessment and risk stratification, and guidelines on disease progression to advanced phase or transformation to acute myeloid leukemia.

The guidelines have been updated to incorporate new clinical, prognostic, morphologic, immunophenotypic, and genetic data and new developments in treatments such as the U.S. Food and Drug Administration approval of fedratinib in 2019 for the treatment of patients with intermediate-risk level 2 or high-risk MF and updated criteria for ruxolitinib failure and intolerance.

“MPN are characterized by a significant symptom burden and a propensity for disease transformation to accelerated and blast phases,” the authors wrote. “The goal of treatment is to reduce symptom burden and the risk of developing thrombotic and hemorrhagic complications. Regular monitoring of disease-related symptoms, assessment of need for cytoreductive therapy, and appropriate evaluation to rule out disease progression should be an integral part of management of patients with MPN.”

Reference

Gerds AT, Gotlib J, Ali H, et al. Myeloproliferative neoplasms, version 3.2022, NCCN clinical practice guidelines in oncology. J Natl Compr Canc Netw. 2022;20(9):1033-1062.