Pathogenesis and Treatment of Anaphylaxis

National Institute Of Allergy And Infectious Diseases

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Abstract

We have evaluated 109 patients referred for idiopathic anaphylaxis (IA) and antigen-specific anaphylaxis (SA) to explore pathogenesis and identify patients with clonal mast cell disease. Overall, 21% have been diagnosed with a clonal mast cell disease, 12.8% with IA, 6.4% with venom anaphylaxis and 1.8% with food or drug-induced anaphylaxis. In addition, 9.3% were diagnosed with alpha-gal syndrome, and 14% were diagnosed with Hereditary alpha-Tryptasemia. Among patients with antigen-induced anaphylaxis, those with venom anaphylaxis in European cohorts have been reported to have a higher prevalence of clonal mast cell disease. In our cohort of patients referred for venom anaphylaxis to date (n=9), seven (78%) have been diagnosed with clonal mast cell disease. Of the nine patients enrolled with food or drug-induced anaphylaxis, thus far, two (22%) have been diagnosed with clonal mast cell disease. In FY 2022, we continued to admit patients with IA or SA as allowed by the Clinical Center COVID restrictions. Most patients are admitted to the inpatient unit and undergo a bone marrow procedure in an attempt to elucidate the etiology and evaluate the pathogenesis of their disease. In collaboration with the NIH Clinical Center's myeloid core facility, we assess all patient bone marrow aspirates and biopsies obtained based on the current WHO criteria to diagnose systemic mastocytosis. In FY 2022, we contributed to the manuscript by Mateja et al with our IA patient population. Insights gained from the anaphylaxis study was important in documenting the variability in baseline tryptase values that improved the sensitivity for confirming the diagnosis of anaphylaxis using event serum tryptase values.