Immune deficiencies, infection, and systemic immune disordersMast cell activation syndrome: A newly recognized disorder with systemic clinical manifestations
Section snippets
Patients
Eighteen adult patients who had characteristic signs and symptoms of MC mediator release were prospectively identified from 2006 to 2009 in an allergy clinic (referred to rule out mastocytosis) and a gastroenterology clinic (referred for unexplained abdominal pain) at a tertiary care center (Brigham and Women’s Hospital, Boston, Mass). This study was designed and initiated several years before 2010, when the current proposed diagnostic guidelines for MCAS by Akin et al15 were published. We used
Patients’ characteristics
We identified 18 patients in our clinics (Table I) with features consistent with the diagnostic criteria for MCAS used in the current study. All but 2 of these patients were women, and age at the time of diagnosis ranged from 20 to 60 years. The most frequent age group represented was 40 to 49 years (44% of patients). Importantly, patients were symptomatic for a mean of 4.6 years (range, 1-9 years) before being given a diagnosis of MCAS. A high incidence of allergies among the patients in our
Discussion
We have identified a group of patients at our tertiary care center with MCAS based on the characteristic symptoms, laboratory evidence of the presence of MC mediators, and response to anti-MC mediator medication. These patients met our inclusion criteria for MCAS, as well as the criteria established by Akin et al.15 Final criteria for this syndrome will have to be established after prospective studies are performed on larger cohorts of patients. Most of our patients had frequent
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Disclosure of potential conflict of interest: C. Akin is a consultant for Novartis and is on the Medical Advisory Board for the Mastocytosis Society. M. C. Castells is a consultant for Merck, receives research support from Ovations for the Cure, and has received funds from the Mastocytosis Society. The rest of the authors have declared that they have no conflict of interest.