title: Mast cell activation syndromes presenting as anaphylaxis.
abstract: Anaphylaxis results from severe systemic mast cell activation. In addition to IgE-mediated and physical triggers, it may occur with a clonal mast cell disease and in an idiopathic fashion without clear provoking factors. Disorders of mast cell activation are classified into primary (clonal), secondary, and idiopathic. Mast cell activation syndrome (MCAS) is a multisystem disorder characterized by objective documentation of elevated mast cell mediators during attacks and a favorable response to antimediator therapy. It should be considered in the differential diagnosis of patients presenting with recurrent anaphylaxis without a clear cause. This article discusses the diagnosis of MCAS.
authors.affiliation: Department of Medicine, Division of Rheumatology, Immunology and Allergy, Mastocytosis Center, Brigham and Women's Hospital, Harvard Medical School, One Jimmy Fund Way, Room 616D Boston, MA 02115, USA. Electronic address: cakin@partners.org.
authors.forename: Cem
authors.initials: C
authors.lastname: Akin
mesh_terms: D000707:Anaphylaxis / Q000175:diagnosis* / Q000276:immunology* / Q000628:therapy; D060965:Clonal Evolution / Q000276:immunology; D003937:Diagnosis, Differential; D019468:Disease Management; D006801:Humans; D007073:Immunoglobulin E / Q000276:immunology; D008407:Mast Cells / Q000276:immunology*; D008415:Mastocytosis / Q000175:diagnosis* / Q000276:immunology* / Q000628:therapy; D013577:Syndrome
chemical_list: D007073:Immunoglobulin E
keywords: Anaphylaxis; Mast cell activation syndrome; Mast cells; Mastocytosis; Tryptase