Recommended management in pre-PMF

Dr Chris Hillis is an assistant professor in the Division of Oncology at McMaster University and hematologist at the Juravinski Hospital and Cancer Centre.

What is the recommended management of prefibrotic primary myelofibrosis (pre-PMF)? Should cytoreductive or antiplatelet treatment be used?

Chris Hillis, MD, MSc: I think in 2019 we should still treat pre-PMF as essential thrombocythemia (ET). This is an emerging condition that we are still trying to understand. Fifteen or 20 years ago these patients would have all been identified as having ET. Now that we have done comparison studies where we have essentially done bone marrows in all patients with myeloproliferative neoplasms (MPNs) as they have been diagnosed, we do have this condition of pre-PMF, which is actually a pretty tricky condition for pathologists to identify because some of the marrow findings can be quite subtle.

But most of the patients, when you are diagnosing them, will still have thrombocytosis and managing them as ET is the best we can do for now. I think the most important thing to remember is that the prognosis is unfortunately not as good as in patients with standard ET and the counseling will be different.