Risk Factor Analysis of Anaphylactic Reactions in Patients With Systemic Mastocytosis

Abstract

Background: Systemic mastocytosis (SM) is a rare disorder of abnormal mast cells in at least 1 extracutaneous organ/tissue. Anaphylaxis is an acute, severe systemic hypersensitivity reaction, and a strong association between SM and anaphylaxis has been shown. However, not all patients with SM experience anaphylaxis. Presently, there are no predictive markers to discriminate patients with SM at high risk of anaphylaxis from those at low risk.

Objective: This study sought to determine risk factors for the occurrence of anaphylaxis in patients with SM.

Methods: A cross-sectional study was conducted in 122 consecutive adult patients with SM admitted to the Mastocytosis Center at Karolinska University Hospital. All patients underwent medical evaluation, including bone marrow biopsy and a thorough allergy workup. To determine risk factors, study subjects were categorized into 2 groups according to the presence (n = 55) or absence (n = 67) of anaphylaxis and compared for their demographic, clinical, and biochemical characteristics.

Results: Patients with SM with anaphylaxis had less frequent presence of mastocytosis in the skin (P < .001), more atopic predisposition (P = .021), higher total IgE levels (P < .001), and lower baseline tryptase levels (27 ng/mL vs 42 ng/mL; P = .024) compared with patients with SM without anaphylaxis.

Conclusions: Patients with SM with anaphylaxis display unique clinical and laboratory features. Hence, a risk analysis tool that is capable of discriminating patients with SM at high risk of anaphylaxis from those at low risk with 86% sensitivity was developed by using the variables male sex, absence of mastocytosis in the skin, presence of atopy, IgE levels of 15 kU/L or more, and baseline tryptase levels of less than 40 ng/mL.

Keywords: Anaphylaxis; Atopy; IgE; Mastocytosis; Risk assessment tool; Risk factors; Tryptase; Venom.