Case Studies in Myeloproliferative Neoplasms - Episode 4

Case 1: Initial Treatment Options for Low Risk P. Vera

Jamile Shammo, MD, reviews initial treatment options for patients with low risk polycythemia vera.

Ruben Mesa, MD: Jamile, let’s start teasing this apart a little. If we’re thinking of a low-risk patient with options such as low-dose aspirin and phlebotomy, what are the goals for treating such an individual and how might we roll that out for a patient?

Jamile M. Shammo, MD: Low-risk patients are typically defined as those who have not had a thrombotic event and those who tend to be younger; essentially below age 60. There you try to go for optimization of the hematologic parameters. Clearly, you need to keep the hematocrit below 45% and maintain the low-dose aspirin. I love your comment about Dr Brady Stein’s study that he talked about. When it comes to outcomes of patients like this, the word is truly optimization. Are we truly taking care of patients who have this entity? Are we doing everything we can to get their disease under control? That is the bottom line. This issue comes to light when you talk about high-risk disease entity.

It’s probably a little easier in low-risk disease because you are capable of doing phlebotomy. Although, in my practice, it’s becoming harder and harder to identify a place where you can do a phlebotomy. Also, do you go by the hemoglobin or do you go by the hematocrit? Every study goes with hematocrit, yet certain places will allow only the hemoglobin to be utilized, and then you’re on your own trying to figure out what type of hemoglobin to go with. The concepts are there but the practical application has a lot of challenges. It would be interesting to delve into the details in practices and see some of the issues they may be dealing with that stand in the way of delivering optimal recommendations. It may be a little easier for low-risk disease patients than it would be for higher-risk individuals with this disease.

Ruben Mesa, MD: Great segue. Prithviraj, why don’t you walk us through the NCCN [National Comprehensive Cancer Network] Guidelines for low-risk polycythemia vera? Before we hear a bit more about your case, how do they pull that together?

Prithviraj Bose, MD: Sure. As Jamile was saying, a low-risk patient is young and has never had a clot. Essentially, the treatment paradigm is still phlebotomy and aspirin. The goal for phlebotomy is usually to keep the hematocrit under 45% based on the CYTO-PV study. Do we ever use cytoreductive therapy in these patients? Absolutely. There could be certain situations where you have to do that for a low-risk patient. That is also addressed by the NCCN Guidelines: things like a new thrombosis or disease-related bleeding, a poor tolerance to phlebotomy, assuming that’s pretty frequent or too frequent for that given individual, splenomegaly, progressive leukocytosis, symptomatic thrombocytosis, and symptoms of the disease that are not controlled by phlebotomy alone. Aspirin generally should be used in all patients. An exception would be those who have high platelet counts leading to you finding evidence of acquired von Willebrand disease, in which case you would not do it. Those are the low-risk guidelines for the most part.

This transcript has been edited for clarity.