Demonstration of an aberrant mast-cell population with clonal markers in a subset of patients with "idiopathic" anaphylaxis

Cem Akin, Linda M. Scott, Can N. Kocabas, Nataliya Kushnir-Sukhov, Erica Brittain, Pierre Noel, Dean D. Metcalfe

Research output: Contribution to journalArticle

154 Citations (Scopus)

Abstract

Idiopathic anaphylaxis remains a perplexing disorder in which existing prophylactic therapy is inadequate. In this prospective study, we sought to determine whether patients with idiopathic anaphylaxis might have evidence for a clonal disorder of mast cells related to mastocytosis and for which novel targeted therapies might be considered. We report 12 patients with "idiopathic" anaphylaxis who did not exhibit either urticaria pigmentosa or the characteristic bone marrow biopsy finding of multifocal mast-cell aggregates observed in systemic mastocytosis. Of these 12 patients, 5 had evidence of 1 or more minor criteria for mastocytosis. C-KIT mutational analysis was positive for the 816D>V activating mutation in 3 of 3 patients in CD25+ bone marrow cells where the analysis was performed. These results demonstrate the presence of an aberrant mast-cell population carrying clonal markers in a subset of patients diagnosed with "idiopathic" anaphylaxis, who may respond to inhibitors targeting mutated C-KIT. This intramural clinical trial was conducted in 2003 and 2004 and was registered at http://clinicalcenter.nih.gov with a study number 03-I-0010. Since the study is now closed, it is no longer available online.

Original languageEnglish (US)
Pages (from-to)2331-2333
Number of pages3
JournalBlood
Volume110
Issue number7
DOIs
StatePublished - Oct 1 2007
Externally publishedYes

Fingerprint

Anaphylaxis
Mast Cells
Bone
Demonstrations
Cells
Biopsy
Mastocytosis
Population
Urticaria Pigmentosa
Systemic Mastocytosis
Bone Marrow Cells
Bone Marrow
Clinical Trials
Prospective Studies
Mutation
Therapeutics

ASJC Scopus subject areas

  • Hematology

Cite this

Akin, C., Scott, L. M., Kocabas, C. N., Kushnir-Sukhov, N., Brittain, E., Noel, P., & Metcalfe, D. D. (2007). Demonstration of an aberrant mast-cell population with clonal markers in a subset of patients with "idiopathic" anaphylaxis. Blood, 110(7), 2331-2333. https://doi.org/10.1182/blood-2006-06-028100

Demonstration of an aberrant mast-cell population with clonal markers in a subset of patients with "idiopathic" anaphylaxis. / Akin, Cem; Scott, Linda M.; Kocabas, Can N.; Kushnir-Sukhov, Nataliya; Brittain, Erica; Noel, Pierre; Metcalfe, Dean D.

In: Blood, Vol. 110, No. 7, 01.10.2007, p. 2331-2333.

Research output: Contribution to journalArticle

Akin, C, Scott, LM, Kocabas, CN, Kushnir-Sukhov, N, Brittain, E, Noel, P & Metcalfe, DD 2007, 'Demonstration of an aberrant mast-cell population with clonal markers in a subset of patients with "idiopathic" anaphylaxis', Blood, vol. 110, no. 7, pp. 2331-2333. https://doi.org/10.1182/blood-2006-06-028100
Akin, Cem ; Scott, Linda M. ; Kocabas, Can N. ; Kushnir-Sukhov, Nataliya ; Brittain, Erica ; Noel, Pierre ; Metcalfe, Dean D. / Demonstration of an aberrant mast-cell population with clonal markers in a subset of patients with "idiopathic" anaphylaxis. In: Blood. 2007 ; Vol. 110, No. 7. pp. 2331-2333.
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