VIDEO: Approaching treatment in myeloproliferative neoplasms varies by patient

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So in MPNs, and this is what I tell my fellows as well, in MPNs, it's not a straight up approach for everyone, every patient is different. And what needs to be addressed in every patient can be different because MPNs or myeloproliferate neoplasms, or chronic myeloid malignancies, which means that they're slow growing evolving myeloid malignancies, which can evolve over months over years and hence affect patients in different ways.

Some of the common features which may not always be seen, but some of the common features that are a feature of MPNs include, for example, splenomegaly, which can be bothersome constitutional symptoms, cytopenias, excess blasts, which are usually a sign of further progression to aggressive versions of this. So that's important to be identified. In early MPNs, like ET, or PV, the idea at least in the immediate future is to prevent the occurrence of a clot. And we have good evidence now that hematocrit control can prevent that in the context of at least PVs.

And the way to think about that is usually my approach is phlebotomy. I do commonly use BESREMi, especially based on the PROUD-PV data, which shows a meaningful reduction of the variant allele frequency of JAK mutation over the course of treatment. Hydrea is something that has stood the test of time and certainly remains an option, but I think my practice is certainly more BESREMi heavy at this time.

In the context of ET again, Pegasus is an option hydrea is an option. A lot of times, monitoring is an option. At least in our practice, we're not always aiming for a very tight playlet control, with the exception of patients who have prior thromboembolic events or have very high platelets, or have concerns or risks of bleeding with very high platelets. So those are those are the patients that we're trying to cytoreduce and usually the agents that are Pegasystems, sometimes hydrea.

Now moving on to myelofibrosis, there's going to be a proportion of your myelofibrosis patients who would be best managed with a wait and watch approach meaning you observe them closely. You see them every two to four months, monitor their blood counts, monitor their spleen, monitor their symptoms, because they were found to have no fibrosis by virtue of one value of mild anemia, which led to a bone marrow biopsy and to the diagnosis, they're not necessarily affected in their day to day life by this diagnosis. The other set of people or the other set of patients is those who are affected and in those, it is important to understand what we're trying to address so low risk patients who are bothered by splenomegaly or who are bothered by constitutional symptoms. JAK inhibitors is the usual go to so we have four JAK inhibitors approved now, which is exciting. We have ruxolitinib, which was the first JAK inhibitor that was approved over a decade ago. We have fedratinib which was approved in 2019, followed by pacritinib a couple of years ago, and now momelotinib as of September last year. Usually for spleen and symptoms, one of the first three, so Jakafi, fedratinib or pacritinib if platelets are less than 50,000 are my go to choices for that.

In patients who also have anemia or have predominantly anemia that needs to be addressed as a part of the clinical complex resulting from other fibrosis, momelotinib has become a common go to with some success and you do see improvements in anemia pretty quickly if it is working.

In patients who are showing evidence of progression. So what that means is they're developing progressive cytopenias or they're developing lack of response or losing their response to JAK inhibitors are those who are showing progression by either rising or progressively rising white count or rising blasts in their blood. Those are patients we tend to add hypomethylating agents to on top of Jakafi and be leading them for transplant at that time, with clear evidence that this is not going to stall for too long and needs transplant for consideration for curative or potentially curative therapy. So that is our usual ballpark approach. But I will again qualify by saying that every patient is different in MPNS, and what we're trying to treat in a given patient is important to understand as we as we try to chalk out a patient's treatment paradigm.