MPN Basics: Defining ET, PV, MF and Associated Symptoms

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At a recent MPN forum in Denver, Dr. Naval Daver from MD Anderson Cancer Center and Dr. Nicholas Di Bella from Rocky Mountain Cancer Centers explained the differences between the essential thrombocythemia (ET), polycythemia vera (PV) and myelofibrosis (MF).  The experts discuss how these conditions are diagnosed and what symptoms, if any, patients may experience.

 

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Please remember the opinions expressed on Patient Power are not necessarily the views of our sponsors, contributors, partners or Patient Power. Our discussions are not a substitute for seeking medical advice or care from your own doctor. That’s how you’ll get care that’s most appropriate for you.

Jeff Folloder:

I'm going to start with you, Dr. Daver.  Help us understand the difference between each of the three types of MPNs.  

Dr. Daver:

Sure.  So thank you all for having me here, and I look forward to a good session today. 

So I think it's actually hard even for medical physicians—and frankly in experts a lot of times—to clearly differentiate between the three.  The easiest way to put it is I think myelofibrosis is an end stage of both PV and ET.  One important thing is I was just speaking to one of our patients here, and you can have ET and PV with fibrosis, but that doesn't necessarily mean you have myelofibrosis, per se.  The diagnosis is really a pathological diagnosis, so we need to get a bone marrow biopsy, we need to get molecular testing, and we need to look at the presence of symptoms, etc., to make that diagnosis.

PV, usually conditionally you have increased red cell production, so some of the older tests, such as red cell indexes, were helpful. But now the JAK2 mutation is probably the best diagnostic test.  So if you have a high hemoglobin, you're having symptoms from that, you're having JAK2 mutation positive with a very low degree of fibrosis, we would usually lean towards PV.  Of course, each individual case has to be looked at.

ET is usually a platelet production problem, so you have too many platelets.  And usually, again, you may have a low degree of fibrosis. But if you don't have significant splenomegaly and the other symptoms of myelofibrosis, weight loss, night sweats, pruritus, then usually we lean more towards ET.  The JAK2 mutation could help, but it's only bothering around 40 to 50 percent of these patients, so it's not clearly diagnostic. 

And myelofibrosis usually is the end stage of PV or ET, and you can have a myelofibrosis that comes on its own without having one of these preexisting type disorders.  Usually these patients have more symptoms.  They have a big spleen about 90 percent of the time.  They have weight loss that is significant, night sweats, itching.  There's usually significant fibrosis that can be seen on the bone marrow by the pathologist, and the JAK2 mutation is positive in about 50 percent of these cases.  I think that's the best way to differentiate them. 

Jeff Folloder:

So, Dr. Di Bella, what are some of the common symptoms that folks might experience with MPNs? 

Dr. Di Bella:

It’s important to emphasize that not all patients have symptoms.  With the routine blood tests that the primary care doctor can do in the office once a year, sometimes we'll find a hematocrit that's very high, 18 or 20—hemoglobin, I'm sorry.  Hematocrit could be in the 60s, or the platelet count may be 800,000 and the patient may have no symptoms.  So that's one category of patients. 

Then, secondly, if the patient's got a high platelet count they may have symptoms of peripheral—peripheral blood supply not working right where their fingers get cold.  They may have headaches.  They may have had a stroke.  Anemia, we all I think are familiar with, shortness of breath, fatigue, dizziness, so those are the main side effects.

When the spleen enlarges patients often will have abdominal plain or loss of an appetite.  What happens as the spleen enlarges it sort of compresses the stomach, and you can't eat what you ordinarily—couldn't eat the same amount you ordinarily would eat.  It's called easy—the medical term for that is called easy satiety. 

So there are a number of symptoms.  Some people will have malaise and fatigue, especially with myelofibrosis.  Polycythemia vera, sometimes we'll see itching that's precipitated by showering or bathing.  Those are the main side—symptoms. 

Please remember the opinions expressed on Patient Power are not necessarily the views of our sponsors, contributors, partners or Patient Power. Our discussions are not a substitute for seeking medical advice or care from your own doctor. That’s how you’ll get care that’s most appropriate for you.

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Page last updated on January 26, 2016