Mastocytosis and insect venom allergy: diagnosis, safety and efficacy of venom immunotherapy

Abstract

The most important causative factor for anaphylaxis in mastocytosis are insect stings. The purpose of this review is to analyse the available data concerning prevalence, diagnosis, safety and effectiveness of venom immunotherapy (VIT) in mastocytosis patients. If data were unclear, authors were contacted personally for further information. Quality of evidence (A: high, B: moderate, C: low and D: very low) and strength of recommendation (strong 1 and weak 2) concerning VIT in mastocytosis patients are assessed according to the Grading of Recommendations Assessment, Development and Evaluation and are marked in square brackets. Results of VIT were described in 117 patients to date. The mean rate of side‐effects during treatment in studies published so far is 23.9% (7.6% requiring adrenaline) with an overall protection rate of 72%. Based on the review we conclude that (1) mastocytosis patients have a high risk of severe sting reactions in particular to yellow jacket, (2) VIT could be suggested [2] in mastocytosis, (3) probably should be done life long [2], (4) VIT in mastocytosis is accompanied by a higher frequency of side‐effects, so (5) special precautions should be taken into account notably during the built up phase of the therapy [2], (6) VIT is able to reduce systemic reactions, but to a lesser extent compared to the general insect venom allergic population [2], so (7) patients should be warned that the efficacy of VIT might be less than optimal and they should continue carrying two adrenaline auto injectors [2].

Adult-onset mastocytosis in the skin is highly suggestive of systemic mastocytosis

“…Even though patients with indolent systemic mastocytosis have an almost normal life expectancy, significant danger may arise from anaphylactic reactions. [27][28][29][30] In a recent study, Brockow et al 30 found a cumulative incidence of anaphylaxis of up to 49% in adults with systemic mastocytosis leading to the recommendation to carry an emergency medication set, including epinephrine. Severe and even fatal intra-and postoperative complications also pose an important threat.…”

How I treat patients with indolent and smoldering mastocytosis (rare conditions but difficult to manage)

“…64,65 Given the likelihood that HVA is IgE mediated in most mastocytosis patients, venom immunotherapy has been proposed as a treatment of choice, albeit with important caveats. 59,[66][67][68] General guidelines for the diagnosis and management of HVA are available, although they are not specific to mastocytosis patients. 69 I pursue full investigation for SM including BM biopsy in patients with anaphylaxis after Hymenoptera stings, regardless of tryptase level or presence of MIS.…”

Diagnosis and Treatment of Anaphylaxis in Patients with Mastocytosis

“…These mediator-targeting drugs are prescribed according to published algorithms [21, 25- blockers (usually IV) are often administered. In patients with mastocytosis in whom an IgE-dependent allergy against hymenoptera venom has been detected, life-long immunotherapy should be performed with recognition of potential side effects, such as increased prevalence of local or systemic allergic reactions [60][61][62][63]. In patients with inhalant allergies and symptoms of allergic rhinitis, the safety and efficacy of immunotherapy has not been systemically studied, but the risk-versus-benefit ratio is generally not considered to be favorable to warrant inhalant immunotherapy, and the treatment plan has to be based on the individual situation in each case.…”