Making the Call in Nonadvanced Systemic Mastocytosis

Nonadvanced systemic mastocytosis (SM) is a group of rare disorders characterized by mast cell accumulation that results in a significant and varied symptomatic burden to the patient. The most prevalent of these symptoms include gastrointestinal symptoms, dermatological symptoms, and recurrent anaphylaxis in response to a variety of triggers such as food, insect venom, and medications. In this Phone-a-Friend series, a multidisciplinary panel of faculty experts will address the importance of high-sensitivity assays to detect the KIT D816V mutation that is responsible for most cases of nonadvanced SM, the recently updated World Health Organization diagnostic criteria, the various triggers of mast cell activation, symptom assessment techniques, and patient education. The faculty will also discuss individualization of symptom-directed treatment strategies, evolving treatment options, and newly approved targeted therapies for the treatment of nonadvanced SM.