Primary Hepatic Diffuse Large B-Cell Lymphoma in a Patient with Scleroderma

Primary Hepatic Lymphoma (PHL) is rare and possibly associated with viral hepatitis and autoimmune diseases. Scleroderma could exceptionally be complicated by lymphoma. We describePHL occurring in a 52-year-old female suffering scleroderma for eight years, with no history of cytotoxic or high-dose glucocorticoid therapy. CT scan, performed to work-up abdominal discomfort, constipation, and elevated alkaline phosphatase, showed a liver mass. Following lefthepatic lobectomy, diffuse large B-cell lymphoma was diagnosed by pathological evaluations.Shortly after operation, chemotherapy began. The patient is alive and free of disease eight yearsafter diagnosis of primary hepatic lymphoma. To the best of our knowledge, this is the first case ofPrimary Hepatic Lymphoma occurring in the setting of long-standing scleroderma. The fact thatour patient had no history of immunosuppressive/ high-dose glucocorticoid therapy may indicatethat similar immunologic abnormalities have pathogenetic role in both scleroderma and non-Hodgkin’s lymphoma.

Keywords:

Liver , Lymphoma , Systemic Scleroderma