Spectrum of mast cell activation disorders.
Mast cell (MC) activation disorders present with multiple symptoms including flushing, pruritus, hypotension, gastrointestinal complaints, irritability, headaches, concentration/memory loss and neuropsychiatric issues. These disorders are classified as: cutaneous and systemic mastocytosis with a c-kit mutation and clonal MC activation disorder, allergies, urticarias and inflammatory disorders and mast cell activation syndrome (MCAS), idiopathic urticaria and angioedema. MCs are activated by IgE, but also by cytokines, environmental, food, infectious, drug and stress triggers, leading to secretion of multiple mediators. The symptom profile and comorbidities associated with these disorders, such as chronic fatigue syndrome and fibromyalgia, are confusing. We propose the use of the term 'spectrum' and highlight the main symptoms, useful diagnostic tests and treatment approaches.
Department of Molecular Physiology and Pharmacology, Molecular Immunopharmacology and Drug Discovery Laboratory, Tufts University School of Medicine and Tufts Medical Center, 136 Harrison Avenue, Boston, MA, USA.
Anastasia I
AI
Petra
Smaro
S
Panagiotidou
Julia M
JM
Stewart
Pio
P
Conti
Theoharis C
TC
Theoharides
D000799:Angioedema / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D000818:Animals; D016207:Cytokines / Q000235:genetics / Q000276:immunology; D015673:Fatigue Syndrome, Chronic / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D005356:Fibromyalgia / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D006801:Humans; D007073:Immunoglobulin E / Q000235:genetics / Q000276:immunology; D008407:Mast Cells / Q000276:immunology* / Q000473:pathology; D034701:Mastocytosis, Cutaneous / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D034721:Mastocytosis, Systemic / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D009154:Mutation; D019009:Proto-Oncogene Proteins c-kit / Q000235:genetics / Q000276:immunology; D014581:Urticaria / Q000235:genetics / Q000276:immunology* / Q000473:pathology
D016207:Cytokines; D007073:Immunoglobulin E; D019009:Proto-Oncogene Proteins c-kit
IgE; antihistamines; brain; c-kit mutation; inflammation; mast cell; mastocytosis; mediators; tryptase