Management of Myeloproliferative Neoplasms: A Focus on Polycythemia Vera and Myelofibrosis - Episode 1

Overview of Myeloproliferative Neoplasms


Harry P. Erba, MD, PhD: Hello, and thank you for joining this OncLive Peer Exchange® titled “Management of Myeloproliferative Neoplasms: A Focus on Polycythemia Vera and Myelofibrosis”. Myeloproliferative neoplasms [MPNs] are often challenging to treat and may require years of therapy and follow-up care. Prognosis is dependent on the specific disorder and response to treatment.

Today, I am joined by a group of my colleagues who are renowned experts in the field of myeloproliferative neoplasm research. During the next 90 minutes, we will discuss evolving research surrounding the treatment of polycythemia vera [PV] and myelofibrosis [MF]. We’ll talk about treatment approaches and highlight emerging agents.

I am Dr Harry Erba, professor of medicine and director of the Leukemia Program at Duke University in Durham, North Carolina.

Participating today on our distinguished panel are:

Dr Rami Komrokji, senior member and professor of oncologic sciences, section head for leukemia and myelodysplastic syndromes [MDS], and vice chair of the Malignant Hematology Department at Moffitt Cancer Center in Tampa, Florida.

Dr Mary Frances McMullin, professor at Queen's University Belfast in Belfast, Northern Ireland.

Dr Ruben Mesa, director of the Mays Cancer Center, Mays Family Foundation Distinguished University Presidential Chair, and professor of medicine at UT Health San Antonio MD Anderson Cancer Center in San Antonio, Texas.

And Dr Jamile Shammo, professor of medicine and pathology and director of the MDS/MPN and Aplastic Anemia Program in the section of hematology at Rush University Medical Center in Chicago, Illinois.

Thank you so much for joining us. Let’s begin. Jamile, could you enlighten us as to what is the real incidence of myeloproliferative neoplasms?

Jamile M. Shammo, MD, FASCP, FACP: Data on epidemiology of myeloproliferative neoplasms have largely been taken either from single institutions or even European data up until a paper in 2016 that reported from the SEER [Surveillance, Epidemiology, and End Results program] data on the incidence of PV, ET [essential thrombocythemia], and myelofibrosis. It revealed something like 10.9 per million for PV, about 9 per million for ET, and about 3 per million for MF. Obviously, these are rare neoplasms, and I think that rarity may pose an issue when it comes to thinking about the diagnosis and making it.

Harry P. Erba, MD, PhD: Ruben, what do the myeloproliferative neoplasms have in common?

Ruben A. Mesa, MD, FACP: They’re clonal neoplasms and they share several features: first, the potential to elevate the blood counts, either the red cells, the white cells, the platelets, or all 3. Second, they have a variable risk of patients developing vascular events, either a thrombosis or bleeding. Third, they clearly can impact patients in terms of developing disease-associated symptoms that we believe are related to a range of difficulties, including elevations in cytokines and mechanical effects from the disease. Next, they have a variable predisposition to enlargement of the spleen that can have consequences in terms of symptoms, as well as discomfort. Finally, they all have a variable risk of progression to a more life-threatening form, whether that be ET and PV progressing to the myelofibrosis phase, or all of them having some variable predisposition toward acute leukemia.

Transcript Edited for Clarity