Mastocytosis is a rare immune disorder that is broken down into two categories:
- cutaneous (CM)
- systemic (SM)
It affects men, women, and children in equal numbers.
Children are more likely to have the cutaneous form which is a benign skin condition, and is likely to resolve during puberty, while adults are usually diagnosed with the systemic form. SM is a chronic, life-long condition that requires ongoing medical care.
But what exactly is mastocytosis?
It’s defined as an abnormal accumulation of mast cells in one or more organs, and treatment is tailored to each individual patient.
Mast cells regulate the body’s response to allergens, and in vast numbers are at the root of inflammation.
People with SM are more prone to potentially fatal anaphylactic shock when exposed to certain toxins and venom–think bee and wasp stings.
Symptoms of mastocytosis can range from mild to severe, and include:
- skin lesions
- gastrointestinal problems
Cognitive issues, or “brain fog,” can often be an issue and can interfere with daily life.
Current treatments include antihistimines, epinephrine, steroids, and cromolyn sodium. In cases in which the disorder is cancerous, chemotherapy may be necessary.
While mastocytosis is incurable, there are new treatments in the clinical trial phase, and one in particular is showing great promise.
A drug called midostaurin performed well enough for the trial to be expanded to the international research community. Researchers are looking to combine midostaurin with other drugs to develop better treatment options for people with advanced mastocytosis.