Abstract
Mast cell activation (MCA) is seen in a variety of clinical contexts and pathologies, including IgE-dependent allergic inflammation, other immunologic and inflammatory reactions, primary mast cell (MC) disorders, and hereditary alpha tryptasemia (HAT). MCA-related symptoms range from mild to severe to life-threatening. The severity of MCA-related symptoms depends on a number of factors, including genetic predisposition, the number and releasability of MCs, organs affected, and the type and consequences of comorbid conditions. In severe systemic reactions, MCA is demonstrable by a substantial increase of basal serum tryptase levels above the individual’s baseline. When, in addition, the symptoms are recurrent, involve more than one organ system, and are responsive to therapy with MC-stabilizing or mediator-targeting drugs, the consensus criteria for the diagnosis of MCA syndrome (MCAS) are met. Based on the etiology of MCA, patients can further be classified as having i) primary MCAS where KIT-mutated, clonal, MCs are detected; ii) secondary MCAS where an underlying IgE-dependent allergy or other reactive MCA-triggering pathology is found; or iii) idiopathic MCAS, where neither a triggering reactive state nor KIT-mutated MCs are identified. Most severe MCA events occur in combined forms of MCAS, where KIT-mutated MCs, IgE-dependent allergies and sometimes HAT are detected. These patients may suffer from life-threatening anaphylaxis and are candidates for combined treatment with various types of drugs, including IgE-blocking antibodies, anti-mediator-type drugs and MC-targeting therapy. In conclusion, detailed knowledge about the etiology, underlying pathologies and co-morbidities is important to establish the diagnosis and develop an optimal management plan for MCAS, following the principles of personalized medicine.
Highlights
Mast cells (MCs) are tissue-fixed effector cells involved in the initiation and perpetuation of allergic inflammation, as well as in a number of other inflammatory states [1,2,3,4,5]
During the preparation of this article, we reviewed other diagnostic criteria and models that have been proposed in the MCA syndrome (MCAS) context in the recent past
MCAS is an unusual MC-induced multi-organ condition characterized by clinical signs and symptoms of anaphylaxis or related pathologies
Summary
Mast cells (MCs) are tissue-fixed effector cells involved in the initiation and perpetuation of allergic inflammation, as well as in a number of other inflammatory states [1,2,3,4,5]. Activated MCs release newly formed cell membrane-derived (lipid-type) mediators of hypersensitivity reactions and cytokines into the extracellular space [1,2,3,4,5]. Severe or even life-threatening MCA may occur when the burden of MCs is high or/and these cells are in a ‘hyperactivated’ state [15,16,17] In such patients, a MCA syndrome (MCAS) may be diagnosed when the symptoms are recurrent and MCAS criteria are fulfilled [15,16,17,18,19,20,21,22,23].
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