Myeloproliferative Neoplasms - Episode 7

MF Prognosis and Common Risks

Drs Shammo and Mesa explain the MF prognosis and possible risk factors.

Ruben Mesa, MD: Now, Jamile, in myelofibrosis [MF], there’s been a tremendous amount of effort to try to understand prognosis. And I think in part because the prognosis symptoms can be wildly different in this disease. There are individuals I’ve seen that have had MF for 40 years without progression. I mean, that’s an outlier but that’s real, and others that from the moment you see them, I mean, they’re really in the middle of a very life-threatening disease. What does prognosis and risk assessment look like in this modern era?

Jamile Shammo, MD, FACP, FASCP: So, as you stated, the disease is rather heterogeneous, and I totally echo Dr Fleischman’s note. Every patient is different, and I think that’s the point of having the international prognostic scoring schema, which would help give the treating physicians an idea about survival of this patient population. There have been various clinical features that have been looked at to get associated with survival. The very first scoring system was published in 2009 and looked at things like hemoglobin, age, and it’s as if every time we have yet another more sophisticated test, then that gets added and tagged onto the prognostic schema.

People realize how important it is to actually develop transfusion dependency, the anemia, and then that kind of get tagged down with the Dynamic International Prognostic Scoring System, which can be applied at any point in time as opposed to at the time of diagnosis as the IPSS [International Prognostic Scoring System] would. The most recent version, published in collaboration with Italian group basically incorporates all of the above. It’s the MIPSS-2 [Mutation-Enhanced International Prognostic Scoring System 2] version [MIPSS70+ v2.0], although I have to say the 1 prior to that the MIPSS70+ was supposed to help us decide or decipher who’s supposed to go to transplant, because again, people who had very high and MIPSS70+ scores, had a very poor survival. You’re talking about for the highest risk, perhaps 2 years, for the lowest risk group versus over a decade for the better prognosis group.

So that’s very important, right? And then again, having next-generation sequencing, having this new cytogenetic grouping that had been recently developed, putting all that together, gives you the MIPPS70+ 2.0. And again, it’s a nice tool to understand the risk for that particular patient and whether or not they ought to be sent to transplant. My fellows do this on every MF patient we see and I find it to be very helpful. The problem is that sometimes you don’t see patients at the time of the diagnosis, and I don’t know how you deal with people who get that referral, does that actually apply?

Ruben Mesa, MD: So I think we have some of both. I’d say that the IPSS, really at the time of diagnosis. Fortunately, many of the newer ones are a bit more dynamic, so they can be applied kind of in real time, as we go. You’re right that it can be a bit of a confusing set of things. I try to share with our fellows that, again, are trying to memorize their prognostic scores for MDS [myelodysplastic syndromes], for myeloma, for a low-grade lymphoma, let alone solid tumors. With prognostic scores, one memorizing them isn’t very helpful. Know that they exist. Know where to find them. And it’ll take some lessons from what are the clinical features that actually have prognostic relevance

Blood counts that makes sense, molecular features—that’s a new part—but that tells us about the biology which molecular features; symptoms, that’s somewhat logical but helpful; age, phenotype of moving toward acute leukemia. We’ve also learned with some of these factors, such as the MYSEC-PM [myelofibrosis secondary to PV and ET prognostic model], other ones that are a bit more specific for individuals that progressed from ET [essential thrombocythemia] or PV [polycythemia vera] because their blood counts can be different. And even now, our colleagues in transplantation have even a different, slightly different scoring system, really, that may be related to transplant outcomes. I would say that the most powerful aspect of prognostic scoring systems in MF is really regarding the urgency of stem cell transplant. I found it to be probably less helpful in terms of helping me decide if I should start therapy.

This transcript has been edited for clarity.