Update Allergy: Gastrointestinal Manifestation in Mastocytosis

The Journal of Allergy and Clinical Immunology Volume 132, Issue 4 , Pages 866-873.e3, October 2013

Gastrointestinal manifestations in mastocytosis: A study of 83 patients

Harry Sokol, Sophie Georgin-Lavialle, Danielle Canioni, Stéphane Barete, Gandhi Damaj, Erinn Soucie, Julie Bruneau, Marie-Olivia Chandesris, Felipe Suarez, Jean-Marie Launay, Achille Aouba, Catherine Grandpeix-Guyodo, Fanny Lanternier. Bernard Grosbois, Christian de Gennes, Pascal Cathébras, Olivier Fain, Nadia Hoyeau-Idrissi, Patrice Dubreuil, Olivier Lortholary, Laurent Beaugerie, Brigitte Ranque, Olivier Hermine,

abstract

Background

Mastocytosis is a heterogeneous disease characterized by mast cell accumulation in 1 or more organs. Gastrointestinal manifestations of systemic mastocytosis have been previously studied in small cohorts of patients, and no specific histologic description is available.

Objective

We sought to assess the clinical and pathologic features of gastrointestinal manifestations in patients with mastocytosis.

Methods

Medical history and gastrointestinal symptoms of patients with mastocytosis (n = 83) were compared with those of matched healthy subjects (n = 83) by means of patient questionnaire. Data were analyzed for epidemiologic, clinical, biological, and genetic factors associated with gastrointestinal symptoms for patients with mastocytosis. A comparative analysis of gastrointestinal histology from patients with mastocytosis (n = 23), control subjects with inflammatory bowel disease (n = 17), and healthy subjects (n = 19) was performed.

Results

The following gastrointestinal symptoms occurred more frequently and were more severe in patients with mastocytosis than in healthy subjects: bloating (33% vs 7.2%, P < .0001), abdominal pain (27.3% vs 4.8%, P < .0001), nausea (23% vs 8.4%, P = .02), and diarrhea (33.85% vs 1.2%, P < .0001). Patients with mastocytosis had a significantly higher incidence of personal history of duodenal ulcer (P = .02). Wild-type (WT) c-Kit was associated with diarrhea (P = .03). Specific histologic lesions were present in patients with mastocytosis but were not correlated with clinical symptoms.

Conclusion

Gastrointestinal manifestations in patients with mastocytosis are highly prevalent and often severe. Clinical symptoms do not correspond to histologic findings, are nonspecific, and can simulate irritable bowel syndrome.

Key words: Mastocytosis, mast cells, c-Kit, gastrointestinal tract

Abbreviations used: MC, Mast cell, SM, Systemic mastocytosis, WT, Wild-type

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