Rami S. Komrokji, MD
A growing understanding about the underlying biology of myelofibrosis has helped improve the diagnosis and treatment of the disease during the past decade and is paving the way for further advancements, according to a panel of experts in the field. “We’ve learned more about the disease biology in the last 5 to 10 years than we did over the 50 years prior, and hopefully, within the next few years, we will have more effective treatments for patients,” Rami S. Komrokji, MD, said during a recent OncLive Peer Exchange®
as a driver mutation in myelofibrosis led to FDA approval in 2011 of ruxolitinib (Jakafi), the first targeted therapy for myelofibrosis. The panel reviewed data from clinical trials of ruxolitinib and discussed other targeted agents in the pipeline. They also talked about important considerations for incorporating ruxolitinib into practice.
Accurately diagnosing myelofibrosis is essential before selecting treatment. In 2016, the World Health Organization (WHO) developed updated criteria for classifying MPNs with the goal of making it easier for clinicians to distinguish between ET, PV, and PMF.3 Srdan Verstovsek, MD, PhD, said the criteria make it clear that strong clinical suspicion is not sufficient to diagnose myelofibrosis. “A combination of tests is required to make the diagnosis,” he said, noting that 2016 WHO guidelines emphasize the necessity of a bone marrow biopsy. Biopsy results in a patient with overt PMF will show proliferation of atypical megakaryocytes accompanied by grade 2 or 3 reticulin and/ or collagen fibrosis. He said other essential tests include genetic screening for a JAK2, CALR,
mutation and a complete blood cell count. Verstovsek said anemia, an elevated serum lactate dehydrogenase level, leukoerythroblastosis in the peripheral smear, leukocytosis, splenomegaly, and constitutional symptoms are all minor criteria used to make a diagnosis.3
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