Gastrointestinal involvement and manifestations in systemic mastocytosis

Abstract

Mastocytosis is a rare and heterogeneous disease characterized by various biological and clinical features with different prognosis and treatments. The disease is usually divided into 2 categories: a pure cutaneous and a systemic disease. Clinical features can be related to mast cells' mediators release or to pathological mast cells infiltration. The diagnosis of mastocytosis is based on clinical, biological, histological, and molecular international criteria. Among all manifestations of the disease, gastrointestinal (GI) symptoms are common and can significantly impair the quality of life. The aim of this article is to review the data regarding GI involvement in mastocytosis. Articles dealing with clinical, pathophysiological, and therapeutic aspects of mastocytosis GI tract involvement were searched for using PubMed. GI manifestations in mastocytosis are reviewed. Pathogenesis of GI symptoms in systemic mastocytosis and their treatment are critically discussed. The most frequent GI symptoms are abdominal pain, diarrhea, nausea, and vomiting. GI lesions may involve all the digestive tract, from the esophagus to the rectum. The histological diagnosis of GI involvement is difficult. The treatment of GI symptoms aims to prevent and limit mast cells degranulation and/or its consequences and more rarely to control tumoral mast cells infiltration. The high prevalence of GI symptoms in mastocytosis and their important functional impact deserves better characterization and treatment in order to improve patients' quality of life. Diagnosis of mastocytosis GI manifestations should be evoked in the case of unexplained severe GI disorders.

Rapid induction of mucosal healing by intensive granulocyte and monocyte adsorptive aphaeresis in active ulcerative colitis patients without concomitant corticosteroid therapy

“…This study is relevant as an approach for chronic constipated patients. Mast cells have been deeply investigated in systemic mastocytosis, 2 and irritable bowel syndrome patients, 3,4 but only a few studies have been published regarding chronic constipation, and most of them are related to Chagas' and Hirschsprung's disease. 5 The pathogenesis of gastrointestinal symptoms in mastocytosis remains unclear; abdominal pain, diarrhoea, peptic disease and malabsorption are the main clinical manifestations in systemic mastocytosis.…”

“…5 The pathogenesis of gastrointestinal symptoms in mastocytosis remains unclear; abdominal pain, diarrhoea, peptic disease and malabsorption are the main clinical manifestations in systemic mastocytosis. 2 Recent investigations have shown that histamine induces the neural hypertrophy and increases mast cells in the gastrointestinal tract. 6 Moreover, mast cells have been recognised as a source of nerve growth factor: in Hirschsprung's disease, mast cells are increased; investigators speculate as a repair phenomenon of the enteral nervous system.…”

“…1 Despite the promising effect of intensive GMAA (twice ⁄ week) on active UC patients 2 , the effect of intensive GMAA in active UC patients without concomitant corticosteroid therapy remains unclear. Recently, we evaluated the effect of ten sessions of intensive, compared with once weekly, GMAA on clinical and endoscopic remission in active UC patients who did not receive corticosteroid therapy, but continued other medications.…”

Flushing Disorders Associated with Gastrointestinal Symptoms: Part 1, Neuroendocrine Tumors, Mast Cell Disorders and Hyperbasophila

“…A number of mechanisms have been postulated to explain the diarrhea including gastric hypersecretion, intestinal villi destruction by increased acidity and prostaglandin D2 oversynthesis causing malabsorption. 71 Gastrointestinal tract symptoms (nausea, vomiting, and abdominal pain) are frequently observed and occur based on the location and density of the mast cell infiltration. The most frequent symptoms are bloating and diarrhea followed by abdominal pain and nausea.…”

Mast Cells and Intestinal Motility Disorders (Mastocytic Enteritis/Colitis)

“…A handful of clinical trials have also reported symptomatic improvement in IBS treated with MC stabilizers [14][15][16][17][18][19][20]. The association between MC and diarrhea/abdominal cramps, is further highlighted by the fact that these symptoms are prevalent in patients with intestinal involvement by systemic mastocytosis [21,22].…”