Treatment There are currently limited treatment options for systemic mastocytosis (SM), and there is no cure for the disease.

For most systemic mastocytosis (SM) patients, the focus of treatment is to control symptoms that are caused by the high levels of histamine and other mast cell-produced mediators. You may be advised to avoid certain dietary and environmental triggers. Anti-allergy medications such as antihistamines are also commonly used to relieve symptoms. Self-injectable epinephrine, often referred to as epi pens, may be prescribed to treat cases of severe allergic reactions.

Advanced System Mastocytosis

For patients with advanced SM, in addition to medications to alleviate symptoms, chemotherapy or steroids may be used to reduce mast cell levels and improve organ function. Only one therapy, imatinib, has been approved for SM by the US Food and Drug Administration, and it is only approved for a small percentage of SM patients with advanced disease who do not have the KIT D816V genetic mutation.

Several investigative medicines designed to target the underlying cause of SM are being studied in clinical trials.