Case 2: SM Treatment Algorithm and Supportive Care Recommendations


Patricia Lugar, MD, MS, shares an allergist’s perspective regarding treatment algorithms and supportive care recommendations for patients with systemic mastocytosis.

Dan DeAngelo, MD, PhD: This patient, the interesting part was that she was followed by her allergist for years and years because there was no therapy before she was put on one of our midostaurin [Rydapt] trials. Everything was tried in terms of trying to help her. Not to put Dr. Lugar on the spot, but you must see some patients, probably not to this extent, doctor, but in terms of your clinical practice, are you often consulted, at least as supportive care? Where would you put this patient? Or have you seen patients like this in your practice?

Patricia Lugar, MD, MS: Yes. We have, but it’s not very common. But I can recollect. I’m thinking about 3 patients that we have seen. One, unfortunately, did go to mast cell leukemia, and they expired shortly after we had a brief consultation. I think they were making the diagnosis at the same time that I was involved in the care. It became clear what the patient had. But absolutely, it’s quite difficult if patients are not associated with a large academic medical center, or if they have very well-intended physicians who are managing the symptoms, it becomes very difficult to want to experiment with other agents like alpha interferon or cladribine, particularly if you don’t have support from oncology. The other 2 patients had come from private practice, allergy/immunology [and] had seen local GI [gastrointestinal specialists]. The same thing; they did have multiple GI biopsies. There was never any mention of mast cell disease. It was food allergies and food intolerance. We could have a many, many hours’ discussion based on that, as you all can imagine. It was very difficult. It was not classic food allergy whatsoever. I agree. You have to think about these disorders. That’s where it begins. Then, you can really zone your evaluation appropriately for the patient. It can really be very, very difficult unless the patient has allergy symptoms, where you would think to measure a tryptase, or has had anaphylaxis. Again, it’s very difficult to know. For example, syncope, particularly in this patient who was quite malnourished. As you can imagine, it could’ve easily been electrolyte disturbances, or a number of other things could’ve been attributed to potential signs that would point you to a mast cell disease. However, actually, I tend to see more indolent cases, but this was a really good pickup to even think to measure the tryptase in this patient because it wouldn’t be.

Dan DeAngelo, MD, PhD: She was followed by GI for a long time, but it was the allergist that ended up picking up the diagnosis and referring her. But it was really amazing.

Transcript edited for clarity.

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