What exactly are myeloproliferative neoplasms (MPNs), and how do doctors diagnose and monitor them over time? MPN expert Dr. Shivani Handa breaks down the differences between essential thrombocytosis (ET), polycythemia vera (PV), and myelofibrosis (MF) and how each affects the body, shares an overview of key testing, and discusses how lab results may affect MPN care.
Dr. Shivani Handa is a hematologist-oncologist at the Ohio State University Comprehensive Cancer Center. Dr. Handa specializes in myeloid malignancies, which includes myeloproliferative neoplasms, myelodysplastic syndrome, and acute leukemias. Learn more about Dr. Handa.
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Transcript
Katherine Banwell:
Dr. Handa, would you start by defining the three myeloproliferative neoplasms, ET, PV, and MF?
Dr. Shivani Handa:
Yeah. So, myeloproliferative neoplasms are chronic blood cancers, and there are three main types. So, there’s ET or essential thrombocytosis, so that’s the condition where your bone marrow produces too much platelets. PV or polycythemia vera is where your bone marrow’s producing too many red blood cells. And then myelofibrosis is the more advanced form of myeloproliferative neoplasms where the bone marrow is scarred. So, fibrosis essentially means scarring. And that leads to actually decrease in the production of blood cells over time. And these patients can be very symptomatic with a lot of symptoms like fatigue, night sweats, weight loss. And they also tend to have an enlarged spleen.
In general, ET and PV are considered more early forms of myeloproliferative neoplasms. Whereas myelofibrosis is more advanced stage.
Katherine Banwell:
Dr. Handa, what key testing should people with MPNs have following a diagnosis?
Dr. Shivani Handa:
So, they should definitely have their complete blood count with a differential checked at diagnosis and at regular intervals. They should, in most cases, have a bone marrow biopsy, and this is especially important in essential thrombocytosis and myelofibrosis cases.
They have to have a bone marrow biopsy to confirm the diagnosis. Especially sometimes essential thrombocytosis can be tricky; it can actually even make this condition called pre-fibrotic myelofibrosis which is a more advanced form.
And we could only tell the difference on a bone marrow biopsy. They would otherwise look similar on blood counts. In case of PV or polycythemia vera, the diagnostic criteria does not necessarily need a bone marrow biopsy if someone has a high enough red blood cell count and they have one of the driver mutations and they have a low erythropoietin level, you could get away without a bone marrow biopsy.
I still do recommend that patient get one just to get a baseline since these things can evolve in the future.
Katherine Banwell:
Dr. Handa, at what point should testing be repeated for someone with an MPN?
Dr. Shivani Handa:
Yeah. So, right now, we repeat testing when something changes. So, either the symptoms change, like if somebody was asymptomatic and is now more symptomatic with fatigue, night sweats, weight loss, etc., or if somebody develops progressive splenomegaly or if their blood counts change.
Like somebody who had a high platelet count with essential thrombocytosis now either has normal platelet count or low platelet counts, that could suggest progression to myelofibrosis.
So, we usually do these when something changes, which I acknowledge is a more reactive approach. I think in the future what we’re trying to develop is a more proactive approach. So, testing these patients at regular intervals like every year to see if something’s changing in their mutation profile that can eventually help intercept early before the disease progresses. But this is not anywhere in the guidelines, this is something that we are hoping to look at prospectively in research and hopefully inform guidelines one day.