Mast cells and mastocytosis
Open accessAbstract
Mast cells have been recognized for well over 100 years. With time, human mast cells have been documented to originate from CD34+ cells, and have been implicated in host responses in both innate and acquired immunity. In clinical immunology, they are recognized for their central role in IgE-mediated degranulation and allergic inflammation by virtue of their expression of the high-affinity receptor for IgE and release of potent proinflammatory mediators. In hematology, the clinical disease of mastocytosis is characterized by a pathologic increase of mast cells in tissues, often associated with mutations in KIT, the receptor for stem cell factor. More recently, and with increased understanding of how human mast cells are activated through receptors including the high-affinity receptor for IgE and KIT, specific tyrosine kinase inhibitors have been identified with the potential to interrupt signaling pathways and thus limit the proliferation of mast cells as well as their activation through immunoglobulin receptors.
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The severity of MCA correlates with the extent of mediator release from MC during an anaphylactic reaction.
“the severity of MCA correlates with the extent of mediator release from MC during an anaphylactic reaction.”
From Proposed Diagnostic Algorithm for Patients with Suspected Mast Cell Activation Syndrome (2019) · cites this paper as [2]
The ‘Mast cells in inflammation’ section identifies mast cells as central to anaphylaxis, and ‘Human mast cell mediators’ describes the mediators they release. Neither section states that the severity of mast-cell activation correlates with the extent of mediator release during an anaphylactic reaction; searches for severity and correlation did not find that relationship. (LLM, conf=0.82)
Experimental — accuracy not yet measuredWell-recognized symptoms of systemic MCA include acute urticaria, flushing, abdominal cramping, diarrhea, hypotensive syncope or near syncope, and tachycardia.
“Well-recognized symptoms of systemic MCA include, among others, acute urticaria, flushing, abdominal cramping, diarrhea, hypotensive syncope or near syncope and tachycardia (Table 3) (1-3,26-28,31).”
From Proposed Diagnostic Algorithm for Patients with Suspected Mast Cell Activation Syndrome (2019) · cites this paper as [2]
The section on diseases of abnormal mast-cell proliferation describes flushing, diarrhea, abdominal pain, and vascular instability as clinical features of mastocytosis. It does not specifically identify acute urticaria, abdominal cramping, hypotensive syncope or near syncope, or tachycardia as symptoms of systemic mast-cell activation; its discussion concerns mastocytosis rather than systemic MCA generally. No page number is available for the passage in our copy. (LLM, conf=0.84)
Experimental — accuracy not yet measuredMCA symptoms are caused by vasoactive and pro-inflammatory mediators released from MC upon activation by allergen via IgER cross-linking or other mechanisms.
“MCA symptoms are caused by several different vasoactive and pro-inflammatory mediators released from MC when these cells are activated by an allergen via IgER cross-linking or other mechanisms (1-5,29-31).”
From Proposed Diagnostic Algorithm for Patients with Suspected Mast Cell Activation Syndrome (2019) · cites this paper as [2]
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- Authors
- Metcalfe, D. D.
- Affiliations
- National Institutes of Health (US)
- Corresponding author
- Metcalfe, Dean D.
- ORCID
- Metcalfe, Dean D.
- Venue
- Blood
- Publisher
- American Society of Hematology
- Published
- 2008-08-15
- Volume
- 112
- Issue
- 4
- Pages
- 946-956
- ISSN
- 0006-4971 (ISSN-L)
- 1528-0020
- PMC
- PMC2515131
- OpenAlex
- W2012512942
- Cited by
- 536
- References
- 112
- Citations by year
- 2026: 9 · 2025: 14 · 2024: 18 · 2023: 21 · 2022: 23 · 2021: 33 · 2020: 27 · 2019: 33 · 2018: 29 · 2017: 39 · 2016: 17 · 2015: 35 · 2014: 55 · 2013: 42 · 2012: 41
- FWCI
- 2.46
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Blood (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Division of Intramural Research, National Institute of Allergy and Infectious Diseases
- National Institutes of Health
- National Institute of Allergy and Infectious Diseases
- NHLBI Division of Intramural Research
- Topics
- Life Sciences › Immunology and Microbiology › Immunology › Mast cells and histamine
- Health Sciences › Medicine › Gastroenterology › Gastrointestinal Tumor Research and Treatment
- Health Sciences › Medicine › Hematology › Platelet Disorders and Treatments
- Keywords
- mast cells, mastocytosis, CD34+ cells, IgE receptor, KIT mutations, stem cell factor, mast cell degranulation, allergic inflammation, tyrosine kinase inhibitors, mast cell activation, innate and adaptive immunity
- MeSH terms
- Animals, Humans, Inflammation, Mast Cells, Mastocytosis, Signal Transduction
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