Mediator-Related Symptoms and Anaphylaxis in Children with Mastocytosis

Abstract

Mastocytosis is characterized by pathological accumulation of mast cells (MC) in various organs. In these patients, MC may degranulate and thereby contribute to clinical symptoms, especially when a concomitant allergy is present. However, MC activation can not only be induced by high-affinity receptors for IgE, but also by anaphylatoxins, neuropeptides, IgG immune complexes, complement-components, drugs, products of bacteria or parasites, as well as physical factors such as heat, cold, vibration, stress, sun, or physical effort. Symptoms due to mediators released by activated MC may develop in adults suffering from systemic mastocytosis, but also evolve in children who usually have cutaneous mastocytosis (CM). Clinically, CM is otherwise characterized by typical brown, maculopapular skin lesions or mastocytoma associated with a positive Darier’s sign. Pruritus and flushing are common and blistering may also be recorded, especially in diffuse CM (DCM). Pediatric patients with mastocytosis may also have gastrointestinal, respiratory, and neurologic complaints. Although anaphylaxis is not a typical finding, pediatric patients with massive skin involvement and high tryptase levels have a relatively high risk to develop anaphylaxis. This paper reviews MC mediator-related symptoms and anaphylaxis in children with mastocytosis, with special emphasis on risk factors, triggers, and management.

Progress of telangiectasia macularis eruptiva perstans exacerbated egg allergy

“…The extent and density of skin lesions as well as higher serum tryptase values were identified as risk factors for anaphylaxis. Furthermore, diffused cutaneous mastocytosis and blistering episodes are risk factors for severe anaphylaxis 5 . Infantile‐onset egg allergy is outgrown with age in general, and this was also observed in the case discussed in this paper.…”

“…Furthermore, diffused cutaneous mastocytosis and blistering episodes are risk factors for severe anaphylaxis. 5 Infantile-onset egg allergy is outgrown with age in general, and this was also observed in the case discussed in this paper. Although the patient's egg allergy might have worsened because of complete elimination of egg products after 8 years Correspondence: Naohiro Itoh, MD PhD, Department of Pediatrics, Faculty of Medical Sciences, University of Fukui, 23-3 Shimoaizuki, Matsuoka, Eiheiji-cho, Yoshida-gun, Fukui 910-1193, Japan.…”

COVID-19 Vaccination in Mastocytosis: Recommendations of the European Competence Network on Mastocytosis (ECNM) and American Initiative in Mast Cell Diseases (AIM)

“…MCs produce and release a large number of biologically active mediators, including vasoactive mediators, coaguloactive substances, and immunoregulatory molecules, such as histamine, heparin, tryptases, prostaglandins, cytokines, or chemokines. 2,5,6 These mediators can cause various symptoms, including headache, fatigue, bone pain, nausea, itching, urticaria, flushing, diarrhea, abdominal discomfort and cramping, hypotension, up to severe anaphylaxis with shock. Symptoms can occur in any variant of mastocytosis and at any time during the course of disease.…”

Mast Cell Activation Syndromes: Collegium Internationale Allergologicum Update 2022

“…In a vast majority of these patients, a somatic mutation in the KIT gene, leading to ligand-independent activation of the receptor and autonomous expansion of MC, is detected and an underlying SM is found [19,[59][60][61][62][63]. Many of these patients suffer from MCA, and some of them even develop an overt MCAS [41,[64][65][66][67][68][69].…”