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Understanding the Myelofibrosis Prognostic Scoring Systems

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Published on August 2, 2021

What Are the Prognostic Scoring Systems for Myelofibrosis?

With so many different myelofibrosis prognostic scoring systems, many of us are left wondering what these scores mean. In this Ask the Expert program, Naveen Pemmaraju, MD, of the MD Anderson Cancer Center, explains the individual variables used to ascertain the status of myelofibrosis and what it could mean going forward.

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Transcript | Understanding the Myelofibrosis Prognostic Scoring Systems

What Are the Different Prognostic Scoring Systems Used to Assess Myelofibrosis?

Dr. Pemmaraju: So one of the great debates in our field is, do you use one of the myelofibrosis scoring systems, and if you do which one? Well, it may come as a surprise to many of our viewers out there, but there's actually almost a dozen, 10 to 12 of these scoring systems that have really come up over the past decade. And so basically the principle of a scoring system is, is there a set of clinical or other easily identified variables about the patient in front of you that you can quickly ascertain and then compare to a hundred or a thousand other patients, and give that patient and yourself some idea of how someone's going to do over time? So let me take you through that quick history because it's actually kind of fascinating.

So in 2009, our colleague [Dr.] Cervantes led a group, this was the first-ever what we call the IPSS, International Prognostic Scoring System, IPSS. And that was about a decade ago now. And the concept there was in over a thousand patients with MF, and remember, this is before the JAK inhibitor era, to try to tell us what the natural history of the disease is. Two huge findings from them. One is that patients can be separated into what's called a low-risk group, hopefully, their survival measured in decades, plus intermediate one and two risk, not as great survivals, and then a more high-risk group. And that there were five variables that could be used and quickly came out of what we call multi-variable analysis, statistical wizardry to just say, here are the five most important factors. And those included at that time age of a certain number, white blood cell count, anemia of a certain number, circulating blasts, and then constitutional symptoms or how the patient feels. But it's a nice nifty system and it really works at the time of diagnosis.

Now, the confusion is since that time, there are all these other scoring systems. There's the DIPSS, so the D-I-P-S-S dynamic, meaning that you can apply it to later on after diagnosis. DIPSS plus, where other variables including cytogenetics were added. And then there's a whole alphabet soup of other scoring systems, MIPSS, GIPSS, et cetera, et cetera. And all of these, what they're trying to do is add in the novel modern data, so molecular information, cytogenetics, bone marrow fibrosis, and the latest scoring systems, whether you have a CALR versus no CALR, the number of mutations, et cetera. And so rather than getting overwhelmed in all this, I think it's nice as a clinician to have all of these tools. I think it's great that groups are trying to put all of them into one comprehensive tool, some of these are available online that anybody can use.

Can Prognostic Scoring Systems Give Us a Better Understanding of Myelofibrosis?

And then importantly, can it help us to understand the patient in front of us, determine therapies, including clinical trial versus available therapies, transplant or not, and give some clue looking into the crystal ball, if you will, of figuring out what's going to happen. Of course, I have to say, as I always do these interviews and think about patients in general–— what applies to a group may not apply to you as an N of one. And so, it's always important to talk to your own doctor, your own provider team and get the clarification in and around these scoring systems because there's other real-world factors that obviously are not captured in these scoring systems. So that's kind of the story, the history of the scoring systems. Let's look at them as one more tool in the journey of understanding your MPN.