Abstract

As of the date of this report, over 25 patients have been evaluated for idiopathic anaphylaxis. All patients were admitted to the inpatient unit and underwent a bone marrow procedure for aspirates and biopsies in an attempt to elucidate the etiology and evaluate the pathogenesis of their disease. Patients also had research blood drawn along with routine labs. In collaboration with the NIH Clinical Center's myeloid core facility, we were able to assess all the patient bone marrow aspirates and biopsies obtained based on the current WHO criteria to rule out the diagnosis of systemic mastocytosis. Mast cells derived from peripheral mononuclear cells were cultured for mast cell growth and activation studies in comparison to mast cells from healthy volunteers. Preliminary data suggests that in some patients with anaphylaxis, their mast cells are more reactive. Six subjects have been enrolled in the randomized treatment protocol with omalizumab (09-I-0129). Data has not yet been analyzed.

  Funding Agency

Agency
National Institute of Health (NIH)
Institute
National Institute of Allergy and Infectious Diseases (NIAID)
Type
Investigator-Initiated Intramural Research Projects (ZIA)
Project #
1ZIAAI001092-03
Application #
8336302
Study Section
Project Start
Project End
Budget Start
Budget End
Support Year
3
Fiscal Year
2011
Total Cost
$463,733
Indirect Cost

  Institution

Name
National Institute of Allergy and Infectious Diseases
Department
Type
DUNS #
City
State
Country
Zip Code

  Publications

Carter, Melody C; Desai, Avanti; Komarow, Hirsh D et al. (2018) A distinct biomolecular profile identifies monoclonal mast cell disorders in patients with idiopathic anaphylaxis. J Allergy Clin Immunol 141:180-188.e3
Carter, M C; Ruiz-Esteves, K N; Workman, L et al. (2018) Identification of alpha-gal sensitivity in patients with a diagnosis of idiopathic anaphylaxis. Allergy 73:1131-1134
Metcalfe, Dean D; Mekori, Yoseph A (2017) Pathogenesis and Pathology of Mastocytosis. Annu Rev Pathol 12:487-514
Muñoz-Cano, Rosa; Pascal, Mariona; Bartra, Joan et al. (2016) Distinct transcriptome profiles differentiate nonsteroidal anti-inflammatory drug-dependent from nonsteroidal anti-inflammatory drug-independent food-induced anaphylaxis. J Allergy Clin Immunol 137:137-146
Boyden, Steven E; Metcalfe, Dean D; Komarow, Hirsh D (2016) Vibratory Urticaria and ADGRE2. N Engl J Med 375:95
Kotarek, Joseph; Stuart, Christine; De Paoli, Silvia H et al. (2016) Subvisible Particle Content, Formulation, and Dose of an Erythropoietin Peptide Mimetic Product Are Associated With Severe Adverse Postmarketing Events. J Pharm Sci 105:1023-7
Lyons, Jonathan J; Yu, Xiaomin; Hughes, Jason D et al. (2016) Elevated basal serum tryptase identifies a multisystem disorder associated with increased TPSAB1 copy number. Nat Genet 48:1564-1569
Kirshenbaum, Arnold S; Cruse, Glenn; Desai, Avanti et al. (2016) Immunophenotypic and Ultrastructural Analysis of Mast Cells in Hermansky-Pudlak Syndrome Type-1: A Possible Connection to Pulmonary Fibrosis. PLoS One 11:e0159177
Metcalfe, Dean D; Pawankar, Ruby; Ackerman, Steven J et al. (2016) Biomarkers of the involvement of mast cells, basophils and eosinophils in asthma and allergic diseases. World Allergy Organ J 9:7
Hox, Valerie; O'Connell, Michael P; Lyons, Jonathan J et al. (2016) Diminution of signal transducer and activator of transcription 3 signaling inhibits vascular permeability and anaphylaxis. J Allergy Clin Immunol 138:187-99

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