PMID- 34292177 OWN - NLM STAT- MEDLINE DCOM- 20211206 LR - 20230208 IS - 1473-6322 (Electronic) IS - 1473-6322 (Linking) VI - 21 IP - 5 DP - 2021 Oct 1 TI - Mast cell activation syndrome: is anaphylaxis part of the phenotype? A systematic review. PG - 426-434 LID - 10.1097/ACI.0000000000000768 [doi] AB - PURPOSE OF REVIEW: Mast cell activation syndrome (MCAS) and anaphylaxis are the result of a spontaneous or triggered pathological degranulation of mast cells (MCs) and might have as substrate normal or pathological MCs (increased burden, aberrant MCs or both). RECENT FINDINGS: This review summarizes the most recent evidence on immunoglobulin E (IgE)-mediated and non IgE-mediated mechanisms underlying MC activation and degranulation and highlights the importance of standardized diagnostic criteria for MCAS. Application of these criteria implies that in most cases the clinical presentation of MCAS meets the diagnostic criteria for anaphylaxis. SUMMARY: Integrating clinical parameters and diagnostic test recognition and underlying clonal MC disease are of utmost importance for a patient-tailored approach. Hereditary alpha-tryptasemia can be encountered in context of anaphylaxis, MCAS and primary MC disorders. CI - Copyright (c) 2021 Wolters Kluwer Health, Inc. All rights reserved. FAU - Sabato, Vito AU - Sabato V AD - University of Antwerp, Faculty of Medicine and Health Sciences, Department of Immunology, Allergology, Rheumatology and the Infla-Med Centre of Excellence. AD - AZ Jan Palfijn Gent, Department of Immunology and Allergology, Ghent, Belgium. FAU - Michel, Moise AU - Michel M AD - Laboratoire d'Immunologie, CHU de Nimes, Nimes. AD - Aix-Marseille Univ, IRD, APHM, MEPHI. AD - IHU Mediterranee Infection, Marseille. FAU - Blank, Ulrich AU - Blank U AD - Universite de Paris, Centre de Recherche sur l'Inflammation, INSERM UMR1149, CNRS ERL8252, Faculte de Medecine site Bichat, Paris, France; Laboratoire d'Excellence Inflamex, Paris, France. FAU - Ebo, Didier G AU - Ebo DG AD - University of Antwerp, Faculty of Medicine and Health Sciences, Department of Immunology, Allergology, Rheumatology and the Infla-Med Centre of Excellence. AD - AZ Jan Palfijn Gent, Department of Immunology and Allergology, Ghent, Belgium. FAU - Vitte, Joana AU - Vitte J AD - Aix-Marseille Univ, IRD, APHM, MEPHI. AD - IHU Mediterranee Infection, Marseille. AD - IDESP, INSERM UMR UA 11, Montpellier, France. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Systematic Review PL - United States TA - Curr Opin Allergy Clin Immunol JT - Current opinion in allergy and clinical immunology JID - 100936359 RN - 37341-29-0 (Immunoglobulin E) SB - IM MH - *Anaphylaxis/diagnosis MH - Humans MH - Immunoglobulin E MH - Mast Cell Activation Syndrome/*diagnosis MH - Mast Cells MH - Phenotype EDAT- 2021/07/23 06:00 MHDA- 2021/12/15 06:00 CRDT- 2021/07/22 12:22 PHST- 2021/07/23 06:00 [pubmed] PHST- 2021/12/15 06:00 [medline] PHST- 2021/07/22 12:22 [entrez] AID - 00130832-202110000-00004 [pii] AID - 10.1097/ACI.0000000000000768 [doi] PST - ppublish SO - Curr Opin Allergy Clin Immunol. 2021 Oct 1;21(5):426-434. doi: 10.1097/ACI.0000000000000768.