Gastrointestinal manifestations of systemic mastocytosis: two case reports
T Csák, K Balassa, A Fodor, A Horvath, J Osztovits, H Eid, Z Nagy, F Szalay, J Demeter · Zeitschrift für Gastroenterologie · 2008 · Heft 05
Bibliografische Angaben
Zusammenfassung
Background: Systemic mastocytosis, clonal disorder of the mast-cell progenitors is associated with activating mutations of the c-kit gene. Gastrointestinal symptoms are present in 80% of patients, mainly due to mast-cell degranulation. On the other hand mast-cells could infiltrate the gastrointestinal tract causing diagnostic problems. The disease may present with hepatosplenomegaly, portal hypertension, cholestasis and/or protein-losing enteropathy. We report two systemic mastocyosis cases with hepatic involvement. Case 1: 68yrs old female patient presented with extreme hepatoslenomegaly, small amount of ascite…