MPN Progression: Overcoming Barriers to Recognizing Warning Signs

Lisa Hatfield:

Another barrier patients may face is knowing when a change in symptoms or test results is simply part of living with an MPN, and when it may signal that the disease needs to be re-evaluated.

What changes in symptoms, blood count, spleen size, or bone marrow findings, or any other factor should patients pay closer attention to?

Dr. Ghaith Abu-Zeinah:

So, you know, weight loss unintentionally. I mean, some people are happy with the weight loss, you know, they, oh, I lost weight, but keep an eye on that, you know, make sure it’s not really a dramatic weight loss that you didn’t intend to have, or if your appetite’s really dropped.

I mean, these are things that you certainly would signal to your physician, we have the ability of kind of keeping an eye on that, too, because we weigh you every time you come in for a visit, and if we notice a major change in your weight, obviously we’d bring that up.

So symptoms really is key for you to monitor, because not only is it an indicator of your disease burden, that doesn’t always mean progression, right? It might be that you have PV, and you’ve been intermittently well…have it well-controlled, and other times maybe it’s just a little more active. So, it doesn’t have to be that you’re progressing or you’re transitioning from PV to MF, but your PV itself could flare up.

So, stay in tune with your symptoms and what’s unusual, you know, whether it be the itching, or the weight loss, or night sweats, or just the fatigue.

And that’s important because actually one of the criteria, the clinical criteria for diagnosing secondary myelofibrosis, so if somebody with ET or PV is actually having progression, symptoms is one of the clinical criterion, or criteria, for diagnosing secondary myelofibrosis.

So, how do you diagnose that progression? Really, you need a bone marrow to confirm that the fibrosis grade has advanced to at least grade 2 out of 3, and then you have to couple that with two clinical features. So, whether it be the symptoms that you report to us, and typically we talk about constitutional symptoms, so specifically the weight loss and the night sweats and maybe fevers in rare instances, and you couple that with another clinical finding.

So what could that be? Anemia, when the hemoglobin trends down. I usually set a cutoff of 10, because 10 is where it’s been looked at, really, as a prognostic indicator. If it drops below 10, it could be a concerning sign.

Nonetheless, we sometimes tend to, while treating PV or ET a little more aggressively, we might overtreat, or the condition itself might get to a point where the clone has regressed enough that you don’t need the same dose of a drug, right?

So, if you stay on the same dose, but you notice your hemoglobin is dropping down, it could just be an effective treatment, and your disease is actually very well-controlled. So, keep an eye on changes in hemoglobin, particularly if it’s less than 10. I don’t usually see that as a you know, hey, the drug dose is too high, because I don’t let it get to that point if it’s from the drug. We often lower the dose and find that we maintain stability over 10 if we’re dealing with ET or PV.

I’ll jump to the [ACT]IVATION tip here, because if you’re going to track signs and symptoms of progression, I would say track your symptoms, track your blood counts for anemia, or even low platelets and track your spleen. So that really is more on your physician. If you really want to take your matter into your own hands, literally speaking, you could, put your hand on your left upper quadrant and look for the spleen.

If you don’t have one to begin with, you’re not going to be expecting anything, but if you do have an enlarged spleen and you actually feel it for one time, then you’ll know how to, you know, keep track of that. But that’s really more on your physician to be monitoring for any changes that could indicate progression.