Gastrointestinal manifestations in mastocytosis: A study of 83 patients

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

Gastrointestinal manifestations in mastocytosis: A study of 83 patients

2013

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.

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