Dr. Mauro on the Risks of Disease Progression in Myelofibrosis

Michael J. Mauro, MD, the leader of the Myeloproliferative Neoplasms Program at Memorial Sloan Kettering Cancer Center, discusses the risks of disease progression in myelofibrosis.

Although disease progression presents differently across the spectrum of myelofibrosis, the threat of transformation to acute myeloid leukemia (AML) remains a significant challenge, Mauro says. The treatment plan of patients who progress to accelerated or blast phase myelofibrosis often needs to be altered in anticipation of AML transformation; however, some approaches will continue patients on their current therapy, such as ruxolitinib (Jakafi), to prevent anti-inflammatory escape or continue clinical benefit, Mauro explains. Often, surveillance for progression in myelofibrosis can identify features of leukemic transformation, such as new mutations in driver genes and sudden changes in allelic fractions.

Additionally, patients with progressive myelofibrosis may experience more profound cytopenias that can indicate more aggressive disease or poor disease control, Mauro says. Other signs of disease progression include splenomegaly, thrombosis, inflammation, generalized organ dysfunction, weight loss, and hypoalbuminemia, Mauro concludes.