title: Disease spectrum in patients with elevated serum tryptase levels.
abstract: BACKGROUND
  Elevated serum tryptase levels can be a sign of mastocytosis, which is a rare disease associated with systemic and/or skin manifestations.
  
  
  OBJECTIVE
  To investigate patients with elevated tryptase levels in regard to their underlying diseases, and to determine whether increased tryptase can be used as a diagnostic marker for underlying mastocytosis.
  
  
  METHODS
  In a retrospective study the data of 96 patients with serum tryptase levels higher than 15 μg/L were systematically analysed. In 48 patients control investigations for baseline tryptase were performed.
  
  
  RESULTS
  Fifty-three of the 96 patients had tryptase levels ≥20 μg/L. A mere 16% of the 96 patients suffered from mastocytosis and had the highest tryptase levels (P < 0.001). The remaining patients had anaphylaxis (36%), urticaria and angioedema (26%), local reactions to insect bites (4%), drug reactions (3%), or miscellaneous diagnoses (15%). Only 16 of these had acute symptoms at tryptase investigation. In all, 48 patients had a follow up; in 7/48 patients with acute symptoms normal tryptase levels were seen at control investigations, but 41/48 (85%) patients showed continuously elevated tryptase levels >15 μg/L and in 30 patients (62%) even values >20 μg/L; 11 of these patients had anaphylaxis, five urticaria, five other diagnoses and nine patients mastocytosis.
  
  
  CONCLUSIONS
  More than 50% of patients with non-mastocytosis such as urticaria and angioedema, drug or anaphylactic reactions repeatedly had tryptase levels higher than 20 μg/L. Since baseline tryptase >20 μg/L is a minor criterion for mastocytosis, these patients should be inspected for skin lesions of mastocytosis and receive a diagnostic body work-up for systemic mastocytosis including a bone marrow biopsy.
authors.affiliation: Department of Dermatology and Venereology, Medical University of Graz, Graz, Austria.
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Aberer
authors.forename: Sandra
authors.initials: S
authors.lastname: Savic
authors.forename: Agnes
authors.initials: A
authors.lastname: Bretterklieber
authors.forename: Harald
authors.initials: H
authors.lastname: Reiter
authors.forename: Andrea
authors.initials: A
authors.lastname: Berghold
authors.forename: Werner
authors.initials: W
authors.lastname: Aberer
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000707:Anaphylaxis / Q000097:blood; D000799:Angioedema / Q000097:blood; D015415:Biomarkers / Q000097:blood; D005260:Female; D006801:Humans; D007299:Insect Bites and Stings / Q000097:blood; D008297:Male; D008415:Mastocytosis / Q000097:blood* / Q000175:diagnosis*; D008875:Middle Aged; D012189:Retrospective Studies; D053802:Tryptases / Q000097:blood*; D014581:Urticaria / Q000097:blood; D055815:Young Adult
chemical_list: D015415:Biomarkers; D053802:Tryptases
keywords: anaphylaxis; mastocytosis; tryptase; urticaria