ArticleThe American journal of surgical pathology2021
Distinct Small Intestine Mast Cell Histologic Changes in Patients With Hereditary Alpha-tryptasemia and Mast Cell Activation Syndrome.
Article in The American journal of surgical pathology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
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Who cites it
18 citing papers in PubMed, 39 citations in OpenAlex.
- Article
- Sexual Dimorphism in Allergic and Mast Cell-Associated Diseases.Clinical reviews in allergy & immunology · 2026Review
- Hereditary α-tryptasemia; a review of mechanisms linking α-tryptase gene dosage to intestinal homeostasis and immunopathology.Frontiers in allergy · 2026Review
- MRGPRX2-expressing mast cells are increased in the GI tract of individuals with active inflammatory bowel disease and hereditary α-tryptasemia.medRxiv : the preprint server for health sciences · 2025Article
- Evolution of mast cell research associated with the Laboratory of Allergic Diseases, 1985-2022: Summary of the 2023 NIAID Symposium.The Journal of allergy and clinical immunology · 2025Review
- Histamine Metabolism in IBD: Towards Precision Nutrition.Nutrients · 2025Review
- MRGPRX2-expressing mast cells are increased in the GI tract of individuals with active inflammatory bowel disease and hereditary α-tryptasemia.Frontiers in allergy · 2025Article
- Hereditary alpha-tryptasemia and monoclonal mast cell disorders.Frontiers in allergy · 2025Review
- From mechanism to management: CEREMAST perspectives on the intersection of HαT and clonal mast cell disorders.Frontiers in allergy · 2025Article
- Non allergic gastrointestinal manifestations of hereditary alpha-tryptasemia.Frontiers in allergy · 2025Review
- Alpha-Tryptase as a Risk-Modifying Factor for Mast Cell-Mediated Reactions.Current allergy and asthma reports · 2024Review
- Addressing Controversies in Mast Cell Activation Syndrome: Analysis Using the Cluster Instrument.Digestive diseases and sciences · 2023Article
- Incorporating Tryptase Genotyping Into the Workup and Diagnosis of Mast Cell Diseases and Reactions.The journal of allergy and clinical immunology. In practice · 2022Review
- Hereditary alpha-tryptasemia modifies clinical phenotypes among individuals with congenital hypermobility disorders.HGG advances · 2022Article
- Clinical relevance of inherited genetic differences in human tryptases: Hereditary alpha-tryptasemia and beyond.Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology · 2021Review
- Small intestinal immunopathology and GI-associated antibody formation in hereditary alpha-tryptasemia.The Journal of allergy and clinical immunology · 2021Article
- Gastrointestinal Manifestations in Systemic Mastocytosis: The Need of a Multidisciplinary Approach.Cancers · 2021Review
- Case Report: Hereditary Alpha Tryptasemia in Children: A Pediatric Case Series and a Brief Overview of Literature.Frontiers in pediatrics · 2021Article
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Authors and funding
12 authors at 5 institutions in 2 countries.
Funding
Abstract
Mast cells (MCs) are important in intestinal homeostasis and pathogen defense but are also implicated in many of the clinical manifestations in disorders such as irritable bowel syndrome. The utility of specific staining for MCs to quantify and phenotype them in intestinal biopsies in patients with gastrointestinal (GI) symptoms is controversial and is not a widely adopted practice. Whether or not intestinal MCs are increased or have a unique phenotype in individuals with hereditary alpha-tryptasemia (HαT), who have extra copies of the MC tryptase gene TPSAB1 and typically elevated baseline serum tryptase levels >8 ng/mL is not known. We examined the duodenal biopsies of 17 patients with HαT and compared them to 15 patients with mast cell activation syndrome who had baseline serum tryptases <8 ng/mL (MCAS-NT) and 12 GI-controls. We determined that the HαT subjects had increased MCs in the duodenum compared with MCAS-NT and GI-controls (median=30.0; interquartile range [IQR]: 20.0 to 40.0 vs. median=15.0; IQR: 5.00 to 20.0; P=0.013 and median=15.0; IQR: 13.8 to 20.0; P=0.004, respectively). These MCs were significantly found in clusters (<15 MCs) and were located throughout the mucosa and submucosa including the superficial villi compared with MCAS-NT and GI-control patients. Spindle-shaped MCs were observed in all groups including controls. These data demonstrate that HαT is associated with increased small intestinal MCs that may contribute to the prevalent GI manifestations observed among individuals with this genetic trait.
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