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Review
. 2021 Jan 30;57(2):124.
doi: 10.3390/medicina57020124.

Mast Cell Activation Disorders

Arianna Giannetti  1 , Emanuele Filice  1 , Carlo Caffarelli  2 , Giampaolo Ricci  1 , Andrea Pession  1
Affiliations
Review

Mast Cell Activation Disorders

Arianna Giannetti et al. Medicina (Kaunas). .

Abstract

Background and Objectives: Mast cell disorders comprise a wide spectrum of syndromes caused by mast cells' degranulation with acute or chronic clinical manifestations. Materials and Methods: In this review article we reviewed the latest findings in scientific papers about mast cell disorders with a particular focus on mast cell activation syndrome and mastocytosis in pediatric age. Results: Patients with mast cell activation syndrome have a normal number of mast cells that are hyperreactive upon stimulation of various triggers. We tried to emphasize the diagnostic criteria, differential diagnosis, and therapeutic strategies. Another primary mast cell disorder is mastocytosis, a condition with a long-known disease, in which patients have an increased number of mast cells that accumulate in different regions of the body with different clinical evolution in pediatric age. Conclusions: Mast cell activation syndrome overlaps with different clinical entities. No consensus was found on biomarkers and no clearly resolutive treatment is available. Therefore, a more detailed knowledge of this syndrome is of fundamental importance for a correct diagnosis and effective therapy.

Keywords: 1,4-methylhistamine; 9 alpha,11 beta- prostaglandin F2α; C-Kit; N-methylhistamine; hereditary alpha-tryptasemia; mast cell; mastocytosis; tryptase; urinary leukotriene E4.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Proposed diagnostic algorithm for MCAS in children. MC(s): mast cell(s); MCAS: mast cell activation syndrome; SM: systemic mastocytosis.
See this image and copyright information in PMC

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