Gastrointestinal manifestations in mastocytosis: a study of 83 patients
Harry Sokol1, Sophie Georgin-Lavialle, Danielle Canioni
1Service de Gastroentérologie et Nutrition, Hôpital Saint-Antoine, AP-HP, Université Pierre et Marie Curie-Paris 6, Paris, France; Equipe AVENIR, Laboratoire INSERM U1057/UMR CNRS 7203, Université Pierre et Marie Curie 6, Paris, France; Equipe Interactions des bactéries commensales et probiotiques avec l'hôte, MICALIS, INRA, Jouy en Josas, France; Centre de Référence des Mastocytoses, Faculté de Médecine et AP-HP Necker-Enfants Malades, Paris, France.
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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