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What do you consider when making a diagnosis of PV?

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When it comes to diagnosis of PV, I think you need to obviously make the accurate diagnosis. Often I see people who come in with PV that were given different diagnosis.

You have to have a very high index of suspicion when you have an individual who presents with a clot at an unusual site or if somebody has a clot plus abnormal blood counts then obviously have to start thinking about MPNs. The problem with MPNs is that sometimes they can present in a way that can overlap with many other benign conditions. So if you have someone who has an autoimmune disorder they could present exactly as that with leukocytosis, thrombocytosis and have a clot related to a different etiology altogether. So all I can say is it has to basically be on the list of differential diagnosis and the physicians might think about it and order the appropriate testing.

I do think that there are certain subtleties to the diagnosis of PV that requires a great deal of expertise, and actually looking at some of the fine-tuning or the certain labs that not everybody looks at, like iron status.

That has a lot to do with people thinking about MPNs as a cause for thrombosis, for example. Often we tend to think about a diagnostic workup when somebody presents with a clot at an unusual location. I think there is a lot to be done about educating community oncologist to think about those disease states and the fact that they can induce thrombosis and hence the necessary tests ought to be ordered because obviously everybody wants to have the correct diagnosis.

I think there's a lot we said about spending time with the patients. So when someone says that the patient is asymptomatic—ok, well what kind of a review of symptoms took place to arrive at that conclusion? At times patients don't necessarily want to share that they have pruritus. They probably think that this isn't something that they need to discuss, or things like abdominal pain or weight loss, whereas the hematologist would know obviously that these are some of the symptoms and signs of MPNs and specifically PV.

It's very important for us to ask specifically and directly about those symptoms one by one to understand.

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.