Belgian Journal of Paediatrics
Mast Cell Activation Syndrome in Children: a Nuanced Approach to Diagnosis and Patient Care
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Keywords

mast cell activation syndrome
mast cell activation disorder
tryptase

Categories

How to Cite

Morelle, C., Fagnard, M., & El Abd, K. (2024). Mast Cell Activation Syndrome in Children: a Nuanced Approach to Diagnosis and Patient Care : A Narrative Review Illustrated by Two Case Reports. Belgian Journal of Paediatrics, 26(4), 283–286. Retrieved from https://belgjpaediatrics.com/index.php/bjp/article/view/305

Abstract

Mast cell activation syndrome (MCAS) covers a range of conditions resulting from recurrent mast cell degranulation, sometimes manifesting as anaphylaxis. It is defined by three criteria: a clinical presentation of mast cell activation (clinical criterion), an increase in tryptase levels (biological criterion), and a favourable response to treatment (treatment criterion). A new classification has been developed that includes mast cell activation disorders (MCAD) that do not strictly meet the above criteria (in particular the biological criterion). This leads to an increasing number of patients being detected, some of whom may however benefit from MCAS treatment. MCAS can be divided into primary (clonal), secondary and idiopathic forms. There are also forms that combine MCAS with mastocytosis, hereditary alpha-tryptasemia and/or allergy. This diagnosis of exclusion needs to be assessed by a specialised multidisciplinary team, to avoid over- and under-diagnosis. Moreover, patients often experience a prolonged medical journey, requiring management of the significant psychosocial and financial repercussions for the child and his family.

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