Aggressive systemic mastocytosis of the liver with cholangitis

Abstract

Mastocytosis is a clonal, neoplastic mast cell proliferation, which is in most cases restricted to the skin (cutaneous mastocytosis), but may infiltrate other organs as well (systemic mastocytosis). Involvement of the liver by a systemic mastocytosis with impairment of liver function has been recognized as sign of an aggressive course of disease (C-finding). This article presents a case of aggressive systemic mastocytosis in a 26-year-old male patient with involvement of the liver mimicking primary sclerosing cholangitis. By histology we could demonstrate multifocal clusters of atypical mast cells infiltrating portal tracts in intimate contact with bile ducts as the cause of cholangitis and liver fibrosis.

Keywords:

• liver
• mastocytosis
• primary sclerosing cholangitis

Informed consent disclosure

The authors state that they have obtained verbal and written informed consent from the patient for the inclusion of their medical and treatment history within the case report.

Financial & competing interests disclosure

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

No writing assistance was utilized in the production of this manuscript.