Since the approval of this protocol, 17 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 comparison studies with healthy volunteers (separate protocol 96-I-0129). There were no adverse events associated with any procedure. No subjects have yet enrolled in the randomized treatment protocol with omalizumab (09-I-0129). We are continuing to screen patients to rule out mastocytosis on the idiopathic anaphylaxis protocol. Thus far, the patients enrolled either have been diagnosed with mastoctyosis (exclusion criteria) or no longer meet frequency data points needed to qualify for the treatment protocol.

Project Start
Project End
Budget Start
Budget End
Support Year
2
Fiscal Year
2010
Total Cost
$243,207
Indirect Cost
City
State
Country
Zip Code
Metcalfe, Dean D; Mekori, Yoseph A (2017) Pathogenesis and Pathology of Mastocytosis. Annu Rev Pathol 12:487-514
Carter, Melody C; Desai, Avanti; Komarow, Hirsh D et al. (2017) A distinct biomolecular profile identifies monoclonal mast cell disorders in patients with idiopathic anaphylaxis. J Allergy Clin Immunol :
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
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
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
Siebenhaar, F; von Tschirnhaus, E; Hartmann, K et al. (2016) Development and validation of the mastocytosis quality of life questionnaire: MC-QoL. Allergy 71:869-77

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