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2017 ; 353
(3
): 207-215
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Characterization of Mast Cell Activation Syndrome
#MMPMID28262205
Afrin LB
; Self S
; Menk J
; Lazarchick J
Am J Med Sci
2017[Mar]; 353
(3
): 207-215
PMID28262205
show ga
BACKGROUND: Mast cell activation syndrome (MCAS), a recently recognized
nonneoplastic mast cell disease driving chronic multisystem inflammation and
allergy, appears prevalent and thus important. We report the first systematic
characterization of a large MCAS population. METHOD: Demographics, comorbidities,
symptoms, family histories, physical examination and laboratory findings were
reviewed in 298 retrospective and 115 prospective patients with MCAS. Blood
samples from prospective subjects were examined by flow cytometry for clonal mast
cell disease and tested for cytokines potentially driving the monocytosis
frequent in MCAS. RESULTS: Demographically, white females dominated. Median ages
at symptom onset and diagnosis were 9 and 49 years, respectively (range: 0-88 and
16-92, respectively) and median time from symptom onset to diagnosis was 30 years
(range: 1-85). Median numbers of comorbidities, symptoms, and family medical
issues were 11, 20, and 4, respectively (range: 1-66, 2-84, and 0-33,
respectively). Gastroesophageal reflux, fatigue and dermatographism were the most
common comorbidity, symptom and examination finding. Abnormalities in routine
laboratories were common and diverse but typically modest. The most useful
diagnostic markers were heparin, prostaglandin D(2), histamine and chromogranin
A. Flow cytometric and cytokine assessments were unhelpful. CONCLUSIONS: Our
study highlights MCAS?s morbidity burden and challenging heterogeneity.
Recognition is important given good survival and treatment prospects.
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