Systemic Mastocytosis

In the 23 to 43% of patients with systemic mastocytosis, the diarrhea is mild to moderate in the majority, with > 90% having a stool volume < 1 L/d. In systemic mastocytosis, the primary cause of the most troubling diarrhea is gastric hypersecretion owing to hyperhistaminemia; therefore, it has a pathogenesis similar to that seen in patients with Zollinger-Ellison syndrome. However, villous atrophy and a secretory component, perhaps owing to prostaglandins,may be important diarrheal factors in some patients with systemic mastocytosis. The diarrhea in these patients is usually controlled by a combination of Hi and H2 receptor antagonists. The mast cell membrane-stabilizing drug cromolyn sodium (disodium chromoglycate) has been reported to be useful to treat diarrhea and other GI symptoms in a small number of patients with systemic mastocytosis. In patients with the malignant forms of mastocytosis, treatment with interferon alpha-2b, as well as chemotherapy and corticosteroids, has been used (Jensen, 2000).

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