Aggressive systemic mastocytosis of the liver with cholangitis

Introduction: Mastocytosis is a clonal, neoplastic mast cell proliferation. It may be divided into cutaneous and systemic mastocytosis. In cutaneous mastocytosis, the course of disease is often indolent. If other organs with or without skin lesions are involved, WHO-criteria for systemic mastocytosis are fulfilled. If organ function is additionally impaired (C-findings) in systemic mastocytosis, an aggressive course of disease can be assumed. Sclerosing cholangitis is a chronic disease of bile ducts in or outside the liver leading to inflammation of bile ducts with obstructive fibrosis and consecutive developmen…