The DOI matches its Crossref record; 30 of the 30 references it cites were found in a registry or an index.
A Clinicopathologic Study of 24 Cases of Systemic Mastocytosis Involving the Gastrointestinal Tract and Assessment of Mucosal Mast Cell Density in Irritable Bowel Syndrome and Asymptomatic Patients
Open accessAbstract
Counting mast cells in gastrointestinal (GI) mucosal biopsies is becoming an increasingly common practice. The primary reason for this exercise is to evaluate for possible involvement by systemic mastocytosis (SM). However, the features of mastocytosis in GI biopsies are not well described. In addition, recent studies have suggested that increased mast cells may be involved in the pathogenesis of some cases of diarrhea-predominant irritable bowel syndrome (IBS); the term "mastocytic enterocolitis" has been proposed for such cases. As the baseline mast cell density in colonic biopsies from normal patients has not been established in large cohorts, there is no widely accepted threshold for what constitutes increased mucosal mast cells. The aims of this study were (1) to determine the utility of GI biopsies for the diagnosis of SM, (2) to characterize the clinical, histologic, and immunohistochemical features of mastocytosis in the GI tract, (3) to determine mast cell density in normal colonic mucosa from a large cohort of asymptomatic patients, and (4) to compare these findings with those from patients with diarrhea-predominant IBS. Twenty-four patients with SM involving the GI tract, 100 asymptomatic patients, and 100 patients with IBS (the latter 2 groups with histologically normal colonic biopsies) were included. For the mastocytosis group, 107 biopsies (70 involved by mastocytosis; 67 mucosal, 3 liver) from 20 women and 4 men were evaluated (median age 59 y). The most commonly involved site was the colon (19 patients, 95%), followed by ileum (86%), duodenum (80%), and stomach (54%). In 16 cases (67%), the first diagnosis of SM was made on the basis of GI biopsies. Seventeen patients had documented cutaneous mastocytosis. Fifteen of 17 patients who underwent bone marrow biopsy had marrow involvement by SM. Eighteen patients had indolent disease, and 6 had aggressive disease (including all 3 with liver involvement). The most common GI symptom was diarrhea, followed by abdominal pain, nausea, weight loss, bloating, vomiting, or reflux. Liver disease presented with hepatomegaly and ascites. Endoscopic abnormalities (observed in 62%) included erythema, granularity, and nodules. Histologically, involved biopsies were characterized by infiltrates of ovoid to spindle-shaped mast cells in aggregates or sheets in the lamina propria, sometimes forming a confluent band underneath the surface epithelium; 25% of biopsies had only focal involvement (single aggregate). Prominent eosinophils were seen in 44% of involved colonic/ileal biopsies and 16% of duodenal biopsies. Mast cells were highlighted by diffuse membranous staining for KIT and CD25. In the nonmastocytosis groups, all biopsies contained singly dispersed mast cells with no aggregates. The mean highest mast cell counts (in a single high-power field) for asymptomatic patients and IBS patients were 26 (range, 11 to 55) and 30 (range, 13 to 59), respectively. In summary, GI (especially colonic) biopsies can establish a diagnosis of SM in patients with GI symptoms. GI involvement is usually subtle and is often associated with prominent eosinophils, which may obscure the mast cell infiltrate. KIT and CD25 are invaluable markers for the diagnosis. Mast cell density in colonic mucosa from asymptomatic patients is highly variable. Although patients with diarrhea-predominant IBS on average have mildly increased mast cells, the overlap in range with that of control patients is too great for this difference to be clinically useful. These findings argue against the utility of counting GI mucosal mast cell in patients with chronic diarrhea.
Overview
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Integrity signals
Registry
Cites
Statistics
Verification
Claims the paper makes
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
The source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
In the citing paper
“However, many reactive in fl ammatory states and neoplasms, as well as individuals with no speci fi c symptoms, may present with an increase in local MCs without MCA and without ful fi lling criteria for MCAS. 133”
From Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review (2021) · cites this paper as [133]
Experimental — accuracy not yet measured
Self-check
Percentages, sums and reported differences re-computed from the paper’s own numbers.
The self-check needs the paper’s full text.
Citations
Cites
Diagnostic criteria and classification of mastocytosis: a consensus proposal
Valent, P., Horny, H., Escribano, L., Longley, B. J., Li, C. Y., Schwartz, L. B., Marone, G., Núñez, R., Akin, C., Sotlar, K., et al. (2001) · Leukemia Research · doi:10.1016/s0145-2126(01)00038-8 · Cited by 1,114
Ma, Y., Zeng, S., Metcalfe, D. D., Akin, C., Dimitrijević, S., Butterfield, J. H., McMahon, G., & Longley, B. J. (2002) · Blood · doi:10.1182/blood.v99.5.1741 · Cited by 444
Self-citationValent, P. (2003) · Leukemia Research · doi:10.1016/s0145-2126(02)00168-6 · Cited by 237
Akin, C., & Metcalfe, D. D. (2004) · Annual Review of Medicine · doi:10.1146/annurev.med.55.091902.103822 · Cited by 215
Mastocytosis: Pathology, genetics, and current options for therapy
Valent, P., Akin, C., Sperr, W. R., Mayerhofer, M., Födinger, M., Fritsche‐Polanz, R., Sotlar, K., Escribano, L., Arock, M., Horny, H., et al. (2004) · Leukemia & lymphoma/Leukemia and lymphoma · doi:10.1080/10428190400010775 · Cited by 196
Zermati, Y., Sepulveda, P. D., Féger, F., Letard, S., Kersual, J., Castéran, N., Gorochov, G., Dy, M., Dumas, A. R., Dorgham, K., et al. (2003) · Oncogene · doi:10.1038/sj.onc.1206120 · Cited by 193
CD25 Indicates the Neoplastic Phenotype of Mast Cells
Sotlar, K., Horny, H., Simonitsch, I., Krokowski, M., Aichberger, K. J., Mayerhofer, M., Printz, D., Fritsch, G., & Valent, P. (2004) · The American Journal of Surgical Pathology · doi:10.1097/01.pas.0000138181.89743.7b · Cited by 173
GASTROINTESTINAL ABNORMALITIES AND INVOLVEMENT IN SYSTEMIC MASTOCYTOSIS
Jensen, R. T. (2000) · Hematology/Oncology Clinics of North America · doi:10.1016/s0889-8588(05)70298-7 · Cited by 160
Aberrant expression of CD30 in neoplastic mast cells in high-grade mastocytosis
Sotlar, K., Cerny‐Reiterer, S., Petat‐Dutter, K., Hessel, H., Berezowska, S., Müllauer, L., Valent, P., & Horny, H. (2010) · Modern Pathology · doi:10.1038/modpathol.2010.224 · Cited by 156
Cited by
Sorted by citations
Items 101–158 of 158
Svrcek, M., & Feakins, R. M. (2020) · Cambridge University Press eBooks · doi:10.1017/9781316823026.009 · Cited by 1
Hu, L., Franco, L., Parikh, J. G., & Zayat, V. (2023) · Cureus · doi:10.7759/cureus.37219 · Cited by 1
Paiva, M. L., Yumeen, S., Saliba, É., & DiMarco, C. (2023) · JAAD Case Reports · doi:10.1016/j.jdcr.2023.04.009 · Cited by 1
Assessment of The Effect of Antidiarrheal Drugs and Plant Extracts on Drosophila melanogaster
Liu, C., & Chassagne, F. (2023) · Journal of Visualized Experiments · doi:10.3791/65877 · Cited by 1
Hamilton, M. J., Greene, L. W., Madigan, L. M., Wang, S. A., Yi, C. A., Kuykendall, A. T., George, T. I., & Castells, M. (2024) · Frontiers in Allergy · doi:10.3389/falgy.2024.1401187 · Cited by 2
Inflammatory Bowel Disease Diagnosis
Feakins, R. M. (2020) · Cambridge University Press eBooks · doi:10.1017/9781316823026.022 · Cited by 1
Mimics of Inflammatory Bowel Disease
Feakins, R. M. (2020) · Cambridge University Press eBooks · doi:10.1017/9781316823026.023 · Cited by 1
Challenging diagnosis of indolent systemic mastocytosis isolated to the GI tract
Horton, L. A., Al-Kourainy, N. K., Kabbani, D., & Bishop, C. R. (2021) · BMJ Case Reports · doi:10.1136/bcr-2020-237268 · Cited by 1
Tryptase Increase without Mastocytosis or Anaphylaxis
Ledford, D. K., & Akin, C. (2017) · The Journal of Allergy and Clinical Immunology In Practice · doi:10.1016/j.jaip.2016.12.017
Case Report Mastocytosis involving rectum: a case report and literature review
Yi, C., Zhang, L.-J., & Israel, B. (2015)
Response to: The Significance of Mast Cell Activation in The Era of Precision Medicine
Schiller, L. R. (2018) · The American Journal of Gastroenterology · doi:10.1038/s41395-018-0358-3
Murray, J. A., Dellon, E. S., Peterson, K. A., Falk, G. W., Chehade, M., Genta, R. M., Gonsalves, N. P., Rothenberg, M. E., Khoury, P., Leung, J., et al. (2019) · The American Journal of Gastroenterology · doi:10.14309/01.ajg.0000594504.91508.61
Gastrointestinal Manifestations of Systemic Mastocytosis
Hamilton, M. J. (2019) · Springer eBooks · doi:10.1007/978-3-030-27820-5_7
Muscularis propria detected at colonic biopsy is not a critical value
Arain, H. A., & Wu, M. L. (2020) · Pathology - Research and Practice · doi:10.1016/j.prp.2020.153045
A Difficult Case of Mastocytic Colitis with Elevated Tryptase
Schussler, E., & Lieberman, P. (2020) · The Journal of Allergy and Clinical Immunology In Practice · doi:10.1016/j.jaip.2020.04.060
Infections of the Oesophagus and Rare Forms of Oesophagitis
Walsh, S. V. (2020) · Cambridge University Press eBooks · doi:10.1017/9781316823026.012
The Value of Gastrointestinal Biopsy
Parisinos, C. A., Sehgal, V., & Parkes, G. C. (2020) · Cambridge University Press eBooks · doi:10.1017/9781316823026.002
Complications of Inflammatory Bowel Disease
Feakins, R. M. (2020) · Cambridge University Press eBooks · doi:10.1017/9781316823026.024
Transplantation, Immunodeficiency, and Immunosuppression
Wong, N. A. C. S. (2020) · Cambridge University Press eBooks · doi:10.1017/9781316823026.005
Radiation and the Gastrointestinal Tract
Feakins, R. M. (2020) · Cambridge University Press eBooks · doi:10.1017/9781316823026.004
Assessment of Gastric Biopsies
Andrews, T., & Campbell, F. (2020) · Cambridge University Press eBooks · doi:10.1017/9781316823026.013
Gastrointestinal Involvement by Systemic Disease
Bateman, A. C. (2020) · Cambridge University Press eBooks · doi:10.1017/9781316823026.003
Walsh, S. V., & Feakins, R. M. (2020) · Cambridge University Press eBooks · doi:10.1017/9781316823026.026
Normal Oesophageal, Gastric and Duodenal Mucosa
Feakins, R. M. (2020) · Cambridge University Press eBooks · doi:10.1017/9781316823026.010
Inflammatory Bowel Disease and the Upper Gastrointestinal Tract
Feakins, R. M. (2020) · Cambridge University Press eBooks · doi:10.1017/9781316823026.017
Normal Lower Gastrointestinal Mucosa
Feakins, R. M. (2020) · Cambridge University Press eBooks · doi:10.1017/9781316823026.018
Geboes, K., & Jouret‐Mourin, A. (2020) · Cambridge University Press eBooks · doi:10.1017/9781316823026.020
Non-Neoplastic Diseases of the Anal Canal
Moorghen, M. (2020) · Cambridge University Press eBooks · doi:10.1017/9781316823026.028
Andrews, T., & Campbell, F. (2020) · Cambridge University Press eBooks · doi:10.1017/9781316823026.014
Úlcera colónica como hallazgo infrecuente de mastocitosis
Cibreiro, C. P., Alonso, M. R., Bueno, J. A. V., & Arriero, G. M. (2020) · Gastroenterología y Hepatología · doi:10.1016/j.gastrohep.2020.06.012
Khakoo, N. S., Rodriguez, A., & Abdalla, S. M. (2020) · The American Journal of Gastroenterology · doi:10.14309/01.ajg.0000710260.33345.25
Eosinophilic, Mastocytic, and Histiocytic Diseases of the Gastrointestinal Tract
Drage, M. G., & Srivastava, A. (2020) · Springer eBooks · doi:10.1007/978-3-030-51268-2_12
Aggressive systemic mastocytosis mimicking lymphoma: a case reportPreprint
Xu, Y., & Gao, J. (2020) · Research Square · doi:10.21203/rs.3.rs-135231/v1
A Vanishing Cecal Mass: A Rare Gastrointestinal Manifestation of Systemic Mastocytosis
Newman, K. M., Vercellotti, G. M., Snover, D. C., Peterson, T. J., & Shmidt, E. (2021) · Cureus · doi:10.7759/cureus.20784
Ho-Yen, C., & Chang, F. (2020) · Cambridge University Press eBooks · doi:10.1017/9781316823026.016
Feakins, R. M. (2020) · Cambridge University Press eBooks · doi:10.1017/9781316823026.001
Clarke, L., & Feakins, R. M. (2020) · Cambridge University Press eBooks · doi:10.1017/9781316823026.015
Colonic ulcer as an uncommon finding of mastocytosis
Cibreiro, C. P., Alonso, M. R., Bueno, J. A. V., & Arriero, G. M. (2021) · Gastroenterología y Hepatología (English Edition) · doi:10.1016/j.gastre.2020.06.026
Mast cell neoplasm: a challenging pathological diagnosis
Natal, R. D. A., Carneiro, H. C., Barra, F. F. D. C., Pagnano, K. B. B., Soares, F. A., & Vassallo, J. (2022) · Human Pathology · doi:10.1016/j.humpath.2022.05.007
Mastocytosis, Gastrointestinal Manifestations of
Lochhead, P. J. (2019) · Elsevier eBooks · doi:10.1016/b978-0-12-801238-3.65664-7
(2016) · Elsevier eBooks · doi:10.1016/b978-0-323-37673-0.50145-2
George, T. I., & Jencks, A. (2020) · Cambridge University Press eBooks · doi:10.1017/9781316535042.012
Czuchlewski, D. R., & George, T. I. (2017) · Molecular pathology library · doi:10.1007/978-3-319-62146-3_9
Feakins, R. M. (2020) · Cambridge University Press eBooks · doi:10.1017/9781316823026.029
Telangiectasia macularis eruptiva perstans with systemic involvement: a case reportPreprint
Keow, J. Y., Ayoub, A. R., Chin-Yee, B. H., Hsia, C. C., Lai, J., Shepherd, J. G., & Robertson, K. (2022) · Research Square · doi:10.21203/rs.3.rs-1768647/v1
Evaluation and diagnosis of mast cell–associated disorders
Park, Y. H., & Lyons, J. J. (2022) · Elsevier eBooks · doi:10.1016/b978-0-323-95061-9.00020-5
Liu, C., & Chassagne, F. (2023) · doi:10.3791/65877-v
Mast cell leukemia- A rare case reported
Patel, D. B., Gandhi, S. H., & Dhruva, G. A. (2024) · Indian Journal of Pathology and Oncology · doi:10.18231/j.ijpo.2024.016
Aggressive systemic mastocytosis: unraveling the mystery behind chronic diarrhea
Chaemsupaphan, T., Weeranawin, P., Kongsakon, R., Nimanong, S., Preechathaveekid, S., Khuhapinant, A., Chalermwai, W. V., & Kaosombatwattana, U. (2025) · Clinical Endoscopy · doi:10.5946/ce.2025.010
Gastritis, Gastropathy, and Ulcer Disease
Advani, R., Rao, S. D., & Liu, Q. Y. (2025) · Elsevier eBooks · doi:10.1016/b978-0-443-11760-2.00026-4
Systemic Mastocytosis Versus Benign Mast Cells
Wen, K. W., & Ohgami, R. S. (2025) · Elsevier eBooks · doi:10.1016/b978-0-323-82688-4.00154-0
Evaluating the Clinical Relevance of Elevated Mucosal Mast Cells in the Gastrointestinal Tract
Damianos, J., Matkowskyj, K. A., Pongdee, T., & Murray, J. A. (2025) · Journal of Clinical Gastroenterology · doi:10.1097/mcg.0000000000002282
Mast Cell Activation Syndrome and Mimickers
Ahn, C., Calle, C. A. G., & Bernstein, J. A. (2026) · Immunology and Allergy Clinics of North America · doi:10.1016/j.iac.2026.01.014
Strategies to detect significant subtle alterations in near-normal gastrointestinal tract samples
Azimpouran, M., Hutchings, D., & Waters, K. M. (2026) · Diagnostic histopathology · doi:10.1016/j.mpdhp.2026.04.003
Aggressive Systemic Mastocytosis Resulting in Hepatic Fibrosis and Non-Cirrhotic Portal Hypertension
Cook, K. H., Singh, B., & Thakral, N. (2026) · Livers · doi:10.3390/livers6050084
Ramírez, G., Khoudari, A., Gonzalez, Y., & Davis-Yadley, A. H. (2026) · ACG Case Reports Journal · doi:10.14309/crj.0000000000002310
Cătană, A., Porr, C., Mondoc, L.-M., Mocanu, L., Olteanu, A. L., Draghila, L., & Iancu, G. M. (2026) · Diagnostics · doi:10.3390/diagnostics16213499
Langreiter, M., Genta, R. M., & Schreiner, P. (2026) · Alimentary Pharmacology & Therapeutics · doi:10.1111/apt.71075
Metadata
- Authors
- Doyle, L. A., Sepehr, G. J., Hamilton, M. J., Akin, C., Castells, M. C., & Hornick, J. L.
- Affiliations
- Brigham and Women's Hospital (US)
- Corresponding author
- Doyle, Leona A.
- Sepehr, Golrokh J.
- Hornick, Jason L.
- Venue
- American Journal of Surgical Pathology
- Published
- 2014-06
- Volume
- 38
- Issue
- 6
- Pages
- 832-843
- ISSN
- 0147-5185 (ISSN-L)
- PMC
- PMC4086834
- OpenAlex
- W1965559358
- Cited by
- 158
- References
- 28
- Citations by year
- 2026: 8 · 2025: 10 · 2024: 7 · 2023: 13 · 2022: 14 · 2021: 15 · 2020: 46 · 2019: 8 · 2018: 10 · 2017: 8 · 2016: 8 · 2015: 8 · 2014: 3
- FWCI
- 4.11
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- The American Journal of Surgical Pathology (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- Funders
- National Institute of Diabetes and Digestive and Kidney Diseases (K08DK094971)
- Topics
- Life Sciences › Immunology and Microbiology › Immunology › Mast cells and histamine
- Health Sciences › Medicine › Gastroenterology › Gastrointestinal motility and disorders
- Health Sciences › Medicine › Gastroenterology › Gastrointestinal Tumor Research and Treatment
- Keywords
- systemic mastocytosis, mast cell density, colonic mucosa, diarrhea-predominant irritable bowel syndrome, mast cell mediators
- MeSH terms
- Adolescent, Adult, Aged, Biopsy, Cell Count, Chronic Disease, Colonoscopy, Diarrhea, Female, Gastrointestinal Diseases, Humans, Immunohistochemistry, Intestinal Mucosa, Male, Mast Cells, Middle Aged, Predictive Value of Tests, Biomarkers, Case-Control Studies, Proto-Oncogene Proteins c-kit, Mastocytosis, Systemic, Irritable Bowel Syndrome, Interleukin-2 Receptor alpha Subunit, Young Adult, Asymptomatic Diseases
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
