PREVENTION MEASURES IN PATIENTS WITH MASTOCYTOSIS – PROTOCOL

Abstract

Mastocytosis comprises a group of rare disorders with diverse clinical presentations, characterized by an abnormal increase number of mast cells in various organs. All patients with mastocytosis are at risk for anaphylaxis due to excessive mast cell mediator release that can occur during anesthesia, administration of radiographic contrast media or after hymenoptera stings. Beside the symptomatic treatment of acute and chronic mast cell-mediator release, and the reduction of the local or systemic mast cell burden, a rational management approach in mastocytosis must include a careful counseling of patients (parents in pediatric cases) and care providers, and avoidance of factors triggering acute mediator release. This protocol forms a pratical guideline for the management of patients with mastocytosis. It includes global preventive measures and measures in specific contexts: local and general anesthesia, administration of radiographic contrast media, and hymenoptera stings. As there are many anaphylaxis triggers in patients with mastocytosis, it is important to have a protocol with guidelines for physicians who are involved in these patients care.