Individual Treatment Plans for MPN Patients

Jamile Shammo, MD, discusses creating individual treatment plans for MPNs and following patients to have a better understanding of how they will respond to treatment.

Bruce Feinberg, DO: We focused a lot on that quality improvement process and how it could be realized to the benefit of patients with MPNs [myeloproliferative neoplasms]. In terms of that journey, I will start with you, Jamile. That journey sounded very fluid. Is it all that fluid, or is it more predictable where you start watching the patient, you see their blood counts, and you could almost say that, within a year, they are going to require this therapy? In Ruben’s conversation before with the comment that biology is not linear, it feels like the curtain can drop at any time. As a practitioner, I felt like I had a bit more insight than that, although you have to be prepared for anything.

In making those decisions, I will give you 1 example that there are rules in solid tumor. In general, once a patient has failed 2 lines of therapy, you start to think pretty predictably about it. In solid tumors, if you fail the first 2 lines of therapy, it is pretty predictable in terms of what their life expectancy is. It is pretty rare that you are going to get significant benefit in third-line treatment or beyond. In diseases like this that are more chronic, are there any predictable points in the care spectrum? What is the expectation that you have going in on how long they are going to have? Do you create a risk profile in which there is low risk, intermediate risk, and high risk? With those low-risk cases, my expectation is 5 years of control with phlebotomy or whatever it may be.

Jamile M. Shammo, MD: I like to separate these into ET [essential thrombocythemia] and PV [polycythemia vera] on 1 hand and then myelofibrosis on the other because, for ET and PV, they are chronic diseases, so the patient’s life expectancy is definitely longer than what you would expect with primary myelofibrosis. Yes, we tend to expect a longer disease state with the first 2 diseases, but I have seen patients with PV go into AML [acute myeloid leukemia] directly without going through a myelofibrotic phase, although that tends to be uncommon. You could predict a chronic course with those 2 entities.

You have to monitor their progression over some period of time. What happens is that the patient as well as the physician can get desensitized because the symptoms’ progression can be insidious and may not necessarily be obvious. People have forgotten to look at peripheral smears, and they do not look at teardrop cells or look at the lymphoblastic picture. They may not look at or feel the spleen to know that the patient with PV who has never had splenomegaly before perhaps has it now—or has leukoerythroblastosis that is moving into a myelofibrotic phase, so it may be time to do a bone marrow biopsy. That is 1 aspect, and we need to do a better job at following those patients chronically.

Let’s now say, if someone moved into that direction, instead of saying, “Let’s give drug A and then drug B,” for patients with myelofibrosis, we should be doing a better job at evaluating their progression risk and what the best treatment plan is from the get-go. That is why you have prognostic scoring schema that we have to assess from the get-go because you have something called stem cell transplantation that has to be considered from the start. You have to decide whether you patient needs to be evaluated for this potentially curative procedure from the get-go. For those patients, now we have a bit more insight into the molecular underpinning of those disorders, and we need to understand this better before we go on simply offering drug A followed by drug B.

I like to lay out a treatment plan for the patients beforehand, anticipating what their survival is going to be. Another point about the progression risk is that it is hugely governed by not only their blood counts but also their comorbidities. If you have someone who may be 75 years old and has every other disease under the sun, they are not going to do as well as somebody else who may be 75 years old, is completely healthy, and has an excellent performance status. That also has to be taken into account.

The bottom line is that we need to do a better job at evaluating every person completely individually and utilizing every possible test that we have for this risk stratification. We then have to do a better job at following those patients as we do with every other hematologic malignancy that we treat, essentially as we do with the low-grade lymphomas, standard lymphomas, intermediate-grade, or what have you. This is something we still do not do; we are lacking in utilizing this when it comes to MPNs.

Transcript edited for clarity.

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