How to Use Prognostic Risk Scoring and Symptom Burden Assessment to Tailor Myelofibrosis Treatment

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Dr. Hobbs:
Hello. This is CME on ReachMD, and I am Dr. Gabriela Hobbs. Today, I'm breaking down the importance of prognostic risk and symptom burden assessment when tailoring treatment for patients with myelofibrosis [MF].

So we have several different prognostic models for myelofibrosis, and they have all built upon each other, starting with the IPSS, followed by the DIPSS, DIPSS-Plus, and MIPSS-70 score that has then been redone, and so we now have the MIPSS70 Version 2. We also have the MYSEC-PM score that was designed specifically for patients with secondary myelofibrosis. These scores are helpful. They use a lot of clinical variables, and the MIPSS score uses molecular variables to define our patients into different groups and to help predict their survival.

In addition to helping to determine the survival of patients and their overall outcome, it can also help to identify patients that need to be referred for a bone marrow transplant evaluation upon their diagnosis.

Survival for patients with myelofibrosis varies by risk, and so patients really are divided into different risk groups. So patients with a low-risk disease can really experience a prolonged survival over a few decades. And patients on the high-risk groups really do have a significantly shortened survival, in the order of 1 to 2 years. And so it is really important to risk stratify our patients to give them a better sense of what to expect, in addition to helping us determine how to better plan for the treatment of our patients.

Now, studies have shown in real-world assessments of physicians treating patients with myelofibrosis, the risk categorization is really not utilized that frequently. Not only is it not utilized that frequently, but it's generally utilized incorrectly, and so many providers assign the incorrect risk category for patients with MF. Patients with underestimated risk are significantly less likely to receive any treatment, and especially to be referred for bone marrow transplantation. And therefore, being aware of these risk scores and utilizing them appropriately is really important for adequate management of patients with MF.

In addition to the different risk factors that patients with MF can have that can impact their survival, patients with myelofibrosis also live with a significant burden of constitutional symptoms. And these can include things like fever, fatigue, early satiety, unintentional weight loss, itching, sexual dysfunction, night sweats, bony pain, and concentration issues.

When taking care of a patient with myelofibrosis, I like to utilize the Myeloproliferative Neoplasm Symptom Assessment Form Total Symptom Score, which is a 10-point questionnaire that easily and quickly evaluates symptoms for patients with myelofibrosis. And it's important to recognize that patients with MF can have a variety of symptoms that don't necessarily correlate and cannot be predicted by what their risk score is based on the risk scores that we were talking about previously.

In addition, it is also important to recognize that patients with myelofibrosis and cytopenias can experience a significant amount of symptoms as well. So studies have demonstrated that patients with thrombocytopenia, especially patients with platelets of less than 100, experienced significant symptoms compared to other patients. And so recognizing that patients in different disease groups and with different blood counts can experience symptoms is also very important to helping to manage these patients.

Some of the symptoms that patients with thrombocytopenia specifically experience are not that different than the other patients with MF, but include fatigue, early satiety, inactivity, night sweats, itching, etc. So it's important to ask our patients, regardless of their blood counts, what their symptoms are.

So in summary, risk stratification is really a key first step when evaluating a patient with myelofibrosis. Risk groups predict survival and also inform therapy decisions. Real-world data suggest that they are underutilized and occasionally used incorrectly. Lastly, cytopenic patients have significant symptom burden.

And that is it, and our time is up. I hope you find the information in this episode helpful, and thank you so much for listening.

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