Biomaterial Database

Mast cell activation syndrome: Myths and realities. 2021

Simin Zhang, and Jonathan A Bernstein

Background: Mast cells (MCs) have been implicated in a spectrum of allergic, immunologic, and infectious inflammatory conditions that involve different organ systems. MC activation can occur through several different surface receptors other than the well known IgE mediated pathway. Methods: We use two representative case reports from our practice to summarize what is currently known about MCAS disorders (reality) so that the clinician can more easily differentiate these conditions from other complex unexplained conditions that are being associated with MC activation (myth). Results: Many complex MC-related conditions, such as clonal MC diseases, have been well characterized but other MC-related disorders, such as MC activation syndrome (MCAS) and idiopathic anaphylaxis, still remain poorly defined. The current consensus recommendations for a diagnosis of MCAS require clinical symptoms of anaphylaxis that correlate with elevation of a MC activation biomarker(s), which improves with H1-antihistamines. Conclusion: Clinical symptoms of MCAS can overlap with other conditions, including neurogenic disorders, e.g., dysautonomia, which necessitate the importance for the clinician to render an accurate diagnosis so that appropriate treatment is provided.

UI	MeSH Term	Description	Entries
D008407	Mast Cells	Granulated cells that are found in almost all tissues, most abundantly in the skin and the gastrointestinal tract. Like the BASOPHILS, mast cells contain large amounts of HISTAMINE and HEPARIN. Unlike basophils, mast cells normally remain in the tissues and do not circulate in the blood. Mast cells, derived from the bone marrow stem cells, are regulated by the STEM CELL FACTOR.	Basophils, Tissue,Basophil, Tissue,Cell, Mast,Cells, Mast,Mast Cell,Tissue Basophil,Tissue Basophils
D008415	Mastocytosis	A rare neoplastic disorder characterized by a clonal proliferation of MAST CELLS, associated with KIT-D816 mutations, and accompanied by aberrant mast cell activation. The abnormal increase of MAST CELLS may occur in only the skin (MASTOCYTOSIS, CUTANEOUS), in extracutaneous tissues involving multiple organs (MASTOCYTOSIS, SYSTEMIC), or in solid tumors (MASTOCYTOMA).
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000090267	Mast Cell Activation Syndrome	A clinically and genetically heterogeneous group of mast cell disorders in which there is aberrant release of mast cell mediators with little to no accompanying proliferation of MAST CELLS.	Idiopathic Mast Cell Activation Disorder,Non-Clonal Mast Cell Activation Disorder,Non-Clonal Mast Cell Activation Syndrome,Nonclonal Mast Cell Activation Syndrome,nc-MCAS,Mast Cell Activation Syndromes,Non Clonal Mast Cell Activation Disorder,Non Clonal Mast Cell Activation Syndrome,nc MCAS
D000707	Anaphylaxis	An acute hypersensitivity reaction due to exposure to a previously encountered ANTIGEN. The reaction may include rapidly progressing URTICARIA, respiratory distress, vascular collapse, systemic SHOCK, and death.	Anaphylactic Reaction,Anaphylactoid Reaction,Anaphylactoid Shock,Shock, Anaphylactic,Anaphylactic Reactions,Anaphylactic Shock,Anaphylactoid Reactions,Reaction, Anaphylactic,Reaction, Anaphylactoid,Shock, Anaphylactoid
D015415	Biomarkers	Measurable and quantifiable biological parameters (e.g., specific enzyme concentration, specific hormone concentration, specific gene phenotype distribution in a population, presence of biological substances) which serve as indices for health- and physiology-related assessments, such as disease risk, psychiatric disorders, ENVIRONMENTAL EXPOSURE and its effects, disease diagnosis; METABOLIC PROCESSES; SUBSTANCE ABUSE; PREGNANCY; cell line development; EPIDEMIOLOGIC STUDIES; etc.	Biochemical Markers,Biological Markers,Biomarker,Clinical Markers,Immunologic Markers,Laboratory Markers,Markers, Biochemical,Markers, Biological,Markers, Clinical,Markers, Immunologic,Markers, Laboratory,Markers, Serum,Markers, Surrogate,Markers, Viral,Serum Markers,Surrogate Markers,Viral Markers,Biochemical Marker,Biologic Marker,Biologic Markers,Clinical Marker,Immune Marker,Immune Markers,Immunologic Marker,Laboratory Marker,Marker, Biochemical,Marker, Biological,Marker, Clinical,Marker, Immunologic,Marker, Laboratory,Marker, Serum,Marker, Surrogate,Serum Marker,Surrogate End Point,Surrogate End Points,Surrogate Endpoint,Surrogate Endpoints,Surrogate Marker,Viral Marker,Biological Marker,End Point, Surrogate,End Points, Surrogate,Endpoint, Surrogate,Endpoints, Surrogate,Marker, Biologic,Marker, Immune,Marker, Viral,Markers, Biologic,Markers, Immune