Mast Cell Activation Syndromes: Collegium Internationale Allergologicum Update 2022.
| Autor: | Valent P; Division of Hematology & Hemostaseology, Department of Internal Medicine I, Medical University of Vienna, Vienna, Austria.; Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria., Hartmann K; Division of Allergy, Department of Dermatology, University Hospital Basel and University of Basel, Basel, Switzerland.; Department of Biomedicine, University Hospital Basel and University of Basel, Basel, Switzerland., Bonadonna P; Allergy Unit, Verona University Hospital, Verona, Italy., Niedoszytko M; Department of Allergology, Medical University of Gdansk, Gdańsk, Poland., Triggiani M; Division of Allergy and Clinical Immunology, University of Salerno, Fisciano, Italy., Arock M; Department of Hematological Biology, Pitié-Salpêtrière Hospital, Pierre et Marie Curie University (UPMC), Paris, France., Brockow K; Department of Dermatology and Allergy Biederstein, Technical University of Munich, Munich, Germany.; Center for Allergy and Environment, Technische Universität und Helmholtz Center Munich, Munich, Germany. |
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| Jazyk: | angličtina |
| Zdroj: | International archives of allergy and immunology [Int Arch Allergy Immunol] 2022; Vol. 183 (7), pp. 693-705. Date of Electronic Publication: 2022 May 23. |
| DOI: | 10.1159/000524532 |
| Abstrakt: | Mast cell activation syndromes (MCASs) are defined by systemic severe and recurrent mast cell activation, usually in form of anaphylaxis, a substantial, event-related increase of the serum tryptase level beyond the individual's baseline and a response of the symptomatology to drugs directed against mast cells, mast cell-derived mediators, or mediator effects. A number of predisposing genetic conditions, underlying allergic and other hypersensitivity states, and related comorbidities can contribute to the clinical manifestation of MCASs. These conditions include hereditary alpha tryptasemia, mastocytosis with an expansion of clonal KIT-mutated mast cells, atopic diathesis, and overt IgE-dependent and IgE-independent allergies. Several of these conditions have overlapping definitions and diagnostic criteria and may also develop concomitantly in the same patient. However, although criteria and clinical features overlap, each of these conditions is characterized by a unique constellation of variables and diagnostic criteria. Since two, three, or more conditions can coexist in the same patient, with obvious clinical implications, it is of crucial importance to diagnose the variant of MCAS precisely and to take all accompanying, underlying and potentially complicating conditions, and comorbidities into account when establishing the management plan. Indeed, most of these patients require multidisciplinary investigations and only a personalized treatment approach can lead to an optimal management plan providing an optimal quality of life and low risk of anaphylaxis. (© 2022 The Author(s). Published by S. Karger AG, Basel.) |
| Databáze: | MEDLINE |
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