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How do you assess the risk of PV progression?

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Long-term effects of polycythemia are things that make my patients worry. Pretty much all of them ask, "What are my chances of developing myelofibrosis and what about leukemia?" It is difficult to live with this disease not knowing that there are specific timelines. We don't know actually what governs disease progression in a given patient, so it's difficult to live with that but again, I think what I've tried to do is educate them on the science of progression—what do we look for, what type of symptoms should bring them back to see me sooner than later—and we always tend to take care of each patient in a very highly individualized fashion.

So there are certain rules and criteria that define very well what disease progression PV is. If you have splenomegaly, for example, or new splenomegaly that hasn't been noted before, well, that means that you have to evaluate the spleen from the get-go in order to determine progression. Similarly, you have to have a baseline for the symptoms that the patients have. You need to know about the blood counts and how they have responded to initiation of treatment and there are also, again, guidelines and specific parameters that have to be met in order for disease progression to be diagnosed.

I think it's very true that monitoring the trend in terms of blood counts is very important. So if I have a patient whose white count has been under control for some time and now all of a sudden they start to have higher counts, and then in the next visit it's even higher, that would be the time actually to take a look at the peripheral smear. What's going on? What type of white cells do we have? Do we have blasts in the peripheral blood? Do we have teardrop cells? Do we have any reason to believe that there's maybe progression to a more advanced disease state at this point? And then, if I do have reason to believe that maybe there is, well then I will think about doing a bone marrow biopsy because this would be the only way actually to demonstrate that you actually have an increase in the amount of fibers or fibrous deposits in the bone marrow. And then they could diagnose myelofibrosis—without that you can get a hint from the peripheral blood—but you don't have confirmation unless you do a bone marrow.

People start to think about progression to myelofibrosis, and in this case I think patients will start to develop anemia; and it's very important to distinguish. Therapeutic anemia resulting from the treatment that we have initiated vs. anemia that comes out of progression to myelofibrosis.

Image of Jamile M Shammo, MD, FASCP, FACP
Jamile M Shammo, MD, FASCP, FACP Associate Professor Rush University Medical Center | Chicago, IL
As principal investigator of clinical trials in her area of expertise, Dr Shammo is heavily involved in education, research, and administrative activities in the Division of Hematology/Oncology. She is recognized nationally for her expertise in bone marrow failure syndromes/paroxysmal nocturnal hemoglobinuria (PNH) and was chosen to serve as a national coordinator for the US PNH registry.