Clonal mast cell disorders in patients with systemic reactions to Hymenoptera stings and increased serum tryptase levels

J Allergy Clin Immunol. 2009 Mar;123(3):680-6. doi: 10.1016/j.jaci.2008.11.018. Epub 2009 Jan 9.

Abstract

Background: Anaphylaxis after Hymenoptera stings has been reported in subjects with mastocytosis, but few data exist regarding disease prevalence in populations allergic to these insects.

Objective: The incidence of clonal mast cell (MC) disorders in subjects with both systemic reactions to Hymenoptera stings and increased serum baseline tryptase (sBT) levels was assessed by using bone marrow (BM) aspirates and biopsy specimens.

Methods: Subjects with a history of a systemic reaction caused by a Hymenoptera sting underwent the standard diagnostic work-up for Hymenoptera allergy, and sBT levels were measured. Subjects with an increased sBT level had BM evaluation that included histology/cytology, flow cytometry, and detection of KIT mutations.

Results: Forty-four (11.6%) of 379 subjects with systemic reactions had increased sBT levels (>11.4 ng/mL), and 31 (70.5%) of these had a history of anaphylaxis. Thirty-four subjects with increased sBT levels underwent a BM analysis. Histology detected diagnostic or subdiagnostic MC infiltrates in 22 (65%) of 34 patients. Abnormal MCs were identified by means of flow cytometry and cytology in 26 (78.8%) of 33 and 20 (58.8%) of 34 subjects, respectively. A KIT mutation was detected in 17 (54.8%) of 31 subjects. The diagnosis was indolent systemic mastocytosis in 21 (61.7%) of 34 subjects and monoclonal MC activation syndrome in 9 (26.5%) of 34 subjects. All subjects with anaphylaxis had one of those 2 disorders.

Conclusion: The concomitant presence of systemic reactions (especially anaphylaxis) after Hymenoptera stings and increased sBT levels strongly suggests that a BM examination is indicated for the diagnosis of clonal MC disease.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Anaphylaxis / etiology*
  • Animals
  • Bone Marrow / pathology*
  • Child
  • Clone Cells / pathology
  • Female
  • Humans
  • Hymenoptera*
  • Incidence
  • Insect Bites and Stings / complications*
  • Male
  • Mast Cells / pathology*
  • Mastocytosis / enzymology
  • Mastocytosis / epidemiology*
  • Mastocytosis / pathology
  • Middle Aged
  • Point Mutation / genetics
  • Point Mutation / immunology
  • Proto-Oncogene Proteins c-kit / genetics
  • Proto-Oncogene Proteins c-kit / immunology
  • Proto-Oncogene Proteins c-kit / metabolism
  • Skin Tests
  • Tryptases / blood*
  • Young Adult

Substances

  • Proto-Oncogene Proteins c-kit
  • Tryptases