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PV Treatment Landscape Balancing Effective Regimens With QOL Challenges

Danielle Bucco
Published: Wednesday, Apr 18, 2018

Jeanne M. Palmer, MD

Jeanne M. Palmer, MD
While there is not a large number of treatments for patients with polycythemia vera (PV), there are currently regimens in the first- and second-line setting that work well for this population, says Jeanne M. Palmer, MD.

on Multiple Myeloma, Palmer, an oncologist in the Bone Marrow Transplant Program at Mayo Clinic, discussed the current treatment landscape for patients with PV and highlighted the impact that ruxolitinib has had on the second-line treatment setting.

OncLive®: Please provide an overview of your presentation on PV.

Palmer: I reviewed the treatment for PV and discussed the diagnosis, different prognostic features, as well as the goals of care and treatment. I covered first-line treatment, which includes either phlebotomy, hydroxyurea, or interferon. The second-line therapy is usually ruxolitinib.

What are some of the biggest challenges in diagnosing patients with PV?

One of the biggest challenges is since the hemoglobin parameters have been lowered, many patients who have met the hemoglobin criteria do not have the JAK2 mutation and the bone marrow biopsy is nonspecific. That is a challenging factor. If they have a JAK2 mutation and a high hemoglobin, then it is a “slam dunk.” However, if they do not and they have the erythrocytosis, it raises the question of whether there is another diagnosis that we are missing. It is possible they could have JAK2-negative PV.

What are we looking for when a patient presents with PV-like symptoms?

We are primarily looking for elevated hemoglobin. You want to see it in the absence of having another reason for it, such as chronic hypoxia, chronic lung disease, or testosterone use. We also look to see if the patient is JAK2 positive. 

Are there any other types of molecular abnormalities other than JAK2 that we are in the process of identifying?

We mostly look for JAK2 mutations. It is either JAK2 or JAK exon 12; there are also some other mutations that can occur in the JAK protein. In other myeloproliferative diseases, we look for calreticulin mutations and MPL mutations, but those are not often seen in PV.
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