Diagnosing Mastocytosis

Описание: Recently, we sat down with Kelli Foster, Co-Chair Pediatric Group of the Mastocytosis Society. In this exclusive interview, Kelli describes mastocytosis and some of the difficulties in getting a diagnosis.
The Mastocytosis Society is a 501(c) 3 nonprofit organization dedicated to supporting patients affected by Mastocytosis or Mast Cell Activation Disorders as well as their families, caregivers, and physicians through research, education, and advocacy.
Mastocytosis is an abnormal accumulation of tissue mast cells in one or more organ systems. Cutaneous mastocytosis is a benign skin disease representing the majority of pediatric cases. In adults, systemic mastocytosis is more common. Skin involvement, typically urticaria pigmentosa, is common in adult patients and can provide an important clue to accurate diagnosis.
Most are managed by a combination of antihistamines or other medications to treat the symptoms. Cormolyn sodium was given orphan drug approval in 1984 to treat mastocytosis and is effective in some patients.
In Canada and Europe, the mast cell stabilizer Zaditen (ketotifen) is available to help some patients. In the United States, ketolifen is only available as eye drops (Zadifor).
For patients with aggressive systemic mastocytosis in adults, the chemotherapy agent Gleevec (imatinib) may be effective and was given orphan drug approval in 2006.