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Mast cell activation syndrome: A newly recognized disorder with systemic clinical manifestations.
MedLine Citation:
PMID:  21621255     Owner:  NLM     Status:  Publisher    
BACKGROUND: Diagnostic criteria for mast cell (MC) activation syndrome have been recently proposed, but clinical studies to validate these criteria are lacking. OBJECTIVE: We sought to determine the clinical manifestations of this newly recognized syndrome in a cohort of patients. METHODS: We prospectively evaluated 18 patients seen at our institution with MC activation syndrome from 2006 to 2009. Patients enrolled had at least 4 of the signs and symptoms of abdominal pain, diarrhea, flushing, dermatographism, memory and concentration difficulties, or headache. Response to treatment with anti-MC mediator medications was assessed based on established criteria. Laboratory tests indicating MC mediator release and histopathology and immunohistochemical studies on gastrointestinal biopsy samples were performed. RESULTS: Ninety-four percent of the patients had abdominal pain, 89% had dermatographism, 89% had flushing, and 72% had the constellation of all 3 symptoms. Patients additionally had headache, diarrhea, and memory and concentration difficulties. All patients had at least 1 positive laboratory test result for an increased MC mediator level. On the basis of the response to treatment criteria, 67% of the patients in the cohort had either a complete or major regression in symptoms while taking medications targeting MC mediators. There was no significant difference in the numbers of intestinal mucosal MCs between our patients and healthy control subjects. CONCLUSION: MC activation syndrome might be the underlying cause of unexplained symptoms when several organ systems are involved, such as the gastrointestinal tract and the skin. It is especially important to be able to recognize the constellation of clinical features because response to anti-MC mediator medications is often excellent.
Matthew J Hamilton; Jason L Hornick; Cem Akin; Mariana C Castells; Norton J Greenberger
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-5-26
Journal Detail:
Title:  The Journal of allergy and clinical immunology     Volume:  -     ISSN:  1097-6825     ISO Abbreviation:  -     Publication Date:  2011 May 
Date Detail:
Created Date:  2011-5-30     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  1275002     Medline TA:  J Allergy Clin Immunol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2011 American Academy of Allergy, Asthma & Immunology. Published by Mosby, Inc. All rights reserved.
Department of Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, Mass.
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