An Introduction to Myeloproliferative Neoplasms


Jamile Shammo, MD, FACP, FASCP, provides an overview of the different types of myeloproliferative neoplasms.

Ruben Mesa, MD:Hello and welcome to this OncLive® Peer Exchange titled Myeloproliferative Neoplasms. I’m Dr Ruben Mesa, the executive director of the Mays Cancer Center at UT Health San Antonio MD Anderson. Joining me in today’s discussions are my colleagues Dr Jamile Shammo from Rush University, Dr Angela Fleischman from the University of California at Irvine and Dr Stephen Oh from Washington University. Thank you all for coming today for today’s discussion.

Today we are going to discuss a number of topics pertaining to the diagnosis and treatment of myeloproliferative neoplasms, with a focus on myelofibrosis. We’ll discuss the latest research in the impact of recent clinical trials on treatment selection and patient management. Let’s get started on our first topic. So first, Jamile, why don’t we start with you? Why don’t you provide an introduction to the myeloproliferative neoplasms? What should folks watching this really think of overall this group of diseases?

Jamile Shammo, MD, FACP, FASCP: The myeloproliferative neoplasms represent a group of clonal hematopoietic stem cell disorders, the most common one of which are polycythemia vera [PV], essential thrombocythemia [ET], and then myelofibrosis. There are 3 others that are less common, and of course there’s always MPN unclassified. There are certain features that join those neoplasms in that they are characterized by myeloproliferation and the clonal nature of this proliferation. They’re also associated with constitutional symptoms, symptoms of splenomegaly and also propensity for thrombosis. And all of them share in the features of the likelihood of progression to a much more serious myeloproliferative disorder. For example, ET/PV will go into myelofibrosis, myelofibrosis will progress to AML [acute myeloid leukemia] essentially, which will be characterized by bone marrow failure and need for transfusions. So practitioners will probably have to keep in mind that for earlier disorders, I should say earlier stages ET/PV, there will be more overriding proliferation that may be characterizing the phenotype of disorder whereas as it progresses it will be more characterized by bone marrow failure and progression to AML.

Ruben Mesa, MD: Very helpful. Now, you used a key phrase in there, neoplasm. Now I know some people watching might have gone through their fellowships when we were still using the term myeloproliferative disorder. When you’re visiting with your patients do you talk to them about this being a malignancy, a cancer, a chronic leukemia, do you find that a bit confusing for them? What’s your approach?

Jamile Shammo, MD, FACP, FASCP: I think that technically speaking the lower-risk MPNs like ET and PV do represent a lower form of malignancy because they do meet the criteria for a malignancy as you have an uncontrolled proliferation of clonal stem cells, for example. So that does meet the criteria for a malignancy. I find that the patients tend to be a little bit more unwelcoming to this type of information or news whereas in myelofibrosis it probably is rather important to sort of emphasize that point because of the nature of the treatments that the patient will likely receive and the fact that they will probably be evaluated at some point for a transplantation.

Ruben Mesa, MD: Very helpful, and I would agree. I tend to find there may be a time and a place to have that conversation with patients and sometimes they’ll ask it but certainly at the time of diagnosis for someone with an earlier disease, it may be really just too much information too soon.

This transcript has been edited for clarity.

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