Preprint Review Version 1 Preserved in Portico This version is not peer-reviewed

Mediator-Related Symptoms and Anaphylaxis in Children with Mastocytosis

Version 1 : Received: 31 January 2021 / Approved: 1 February 2021 / Online: 1 February 2021 (11:36:31 CET)

A peer-reviewed article of this Preprint also exists.

Brockow, K.; Plata-Nazar, K.; Lange, M.; Nedoszytko, B.; Niedoszytko, M.; Valent, P. Mediator-Related Symptoms and Anaphylaxis in Children with Mastocytosis. Int. J. Mol. Sci. 2021, 22, 2684. Brockow, K.; Plata-Nazar, K.; Lange, M.; Nedoszytko, B.; Niedoszytko, M.; Valent, P. Mediator-Related Symptoms and Anaphylaxis in Children with Mastocytosis. Int. J. Mol. Sci. 2021, 22, 2684.

Journal reference: Int. J. Mol. Sci. 2021, 22, 2684
DOI: 10.3390/ijms22052684

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.

Keywords

mast cells; mastocytosis; mediator-related symptoms; anaphylaxis; children

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