Additional Considerations


There is great heterogeneity in the presentation of mastocytosis and mast cell activation syndromes (MCAS). Some patients present with no or very few symptoms, while others are almost completely debilitated by chronic symptoms that require a multitude of medications to control. Some factors that can impact presentation include whether or not the disease is neoplastic, the location of mast cells involved in the disease, and the specific triggers that initiate mast cell activation in a given patient. For example, a patient for whom environmental heat is a trigger may be very symptomatic daily if they live in a warm climate. In some patients, combinations of triggers can produce acute symptoms, such as when exercising on a hot day after eating a high histamine food, like a tomato. Co-existing conditions, for example, allergy or autoimmune diseases, can also have a significant effect on the presentation and course of mast cell disorders. Anaphylaxis, a life-threatening event, is always a powerful presenting symptom that requires comprehensive evaluation.

Patients may need to be treated by physicians from multiple specialties, depending on disease type, signs, symptoms and prognosis. Allergists/immunologists, dermatologists, hematologists, gastroenterologists, cardiologists, endocrinologists, neurologists, psychiatrists, psychologists, orthopedists, anesthesiologists, emergency room physicians, allied health professionals, and others, may all play a vital role for patients with MCDs. Communication between specialists is absolutely critical for positive patient outcomes.