From the Guidelines
Gastrointestinal symptoms of mastocytosis can be prominent, with abdominal pain and diarrhea occurring in up to 40% of children, as reported in a study published in the American Journal of Clinical Dermatology 1. These symptoms are thought to be due to the local release of mast cell mediators, such as histamine, leukotrienes, and prostaglandins, which can cause a range of gastrointestinal issues. Some key points to consider include:
- Abdominal pain and diarrhea are common gastrointestinal symptoms of mastocytosis, affecting up to 40% of children 1
- Hyperacidity and peptic ulcers have been reported, although peptic ulcer disease is rare in children 1
- Management of gastrointestinal symptoms typically involves a combination of medications, such as H1 and H2 antihistamines, proton pump inhibitors, and mast cell stabilizers
- Avoiding known triggers and following a low-histamine diet may also be beneficial in managing symptoms
- Prompt recognition and treatment of gastrointestinal symptoms are essential to improve quality of life and prevent complications. In terms of specific treatment options, H1 and H2 antihistamines, such as cetirizine and famotidine, may be effective in managing gastrointestinal symptoms, as well as proton pump inhibitors like omeprazole and mast cell stabilizers like oral cromolyn sodium 1.
From the FDA Drug Label
Four randomized, controlled clinical trials were conducted with Cromolyn Sodium Oral Solution (Concentrate) in patients with either cutaneous or systemic mastocytosis; ... Clinically significant improvement in gastrointestinal symptoms (diarrhea, abdominal pain) were seen in the majority of patients
- Gastrointestinal symptoms of mastocytosis, such as diarrhea and abdominal pain, can be improved with Cromolyn Sodium Oral Solution (Concentrate) in the majority of patients 2.
From the Research
Gastrointestinal Symptoms of Mastocytosis
- The most frequent gastrointestinal (GI) symptoms in mastocytosis are abdominal pain, diarrhea, nausea, and vomiting 3, 4, 5.
- GI lesions may involve all parts of the digestive tract, from the esophagus to the rectum 3.
- The histological diagnosis of GI involvement in mastocytosis is difficult, and specific histologic lesions may not correlate with clinical symptoms 3, 5.
- Gastrointestinal manifestations in patients with mastocytosis are highly prevalent and often severe, and can simulate irritable bowel syndrome 5.
Pathogenesis and Treatment of GI Symptoms
- GI symptoms in mastocytosis are largely due to the release of mast cell mediators, but may also be due to other factors such as tumoral mast cell infiltration 3, 4.
- Treatment of GI symptoms aims to prevent and limit mast cell degranulation and/or its consequences, and may involve a combination of therapies including H1 and H2 antihistamines, leukotriene receptor blockade, and inhibition of mediator synthesis 4, 6, 7.
- Patients with advanced mastocytosis may experience signs and symptoms of gastrointestinal dysfunction due to massive infiltration of clonal mast cells in the tissues, and may require more aggressive treatment including reduction of mast cell numbers 4, 7.