A Case of Secondary Sclerosing Cholangitis in the setting of Non-Hodgkin’s Lymphoma

Abstract

Sclerosing cholangitis represents a spectrum of cholestatic liver disease characterized by inflammation, fibrosis, and stricture of the bile ducts. A 67-year-old Caucasian female with a history of breast cancer in remission, presented with jaundice and an exophytic mass at the base of the tongue. Laboratory data revealed cholestasis with alkaline phosphatase 953 U/L, total bilirubin 7.7 mg/dL, direct bilirubin 6.4 mg/dL, and gamma-glutamyltransferase 3369 U/L. Computed tomography (CT) scan showed widespread lymphadenopathy in the chest, abdomen, and pelvis concerning for lymphoma, acute pancreatitis and biliary dilation with hyperenhancement of the common bile duct wall. Diffuse intrahepatic biliary ductal dilatation and narrowing with multifocal stenosis of the proximal and distal aspects of the common bile duct was seen on magnetic resonance cholangiopancreatography (MRCP). Findings were consistent with sclerosing cholangitis. Pathology of the oral lesion revealed activin receptor-like kinase 1 (ALK1) positive anaplastic large cell lymphoma. Chemotherapy was initiated with cyclophosphamide, doxorubicin, adriamycin, vincristine, etoposide, and prednisone (CHOEP-14) regimen, which resulted in significant clinical improvement along with a remarkable decrease in the liver function tests. Non-Hodgkin’s lymphoma (NHL) has only rarely been reported in the literature as a cause of secondary sclerosing cholangitis, i.e., only 0.2% to 2.0% of patients with NHL present with biliary tract obstruction. It is essential for gastroenterologists, oncologists, and radiologists to recognize sclerosing cholangitis occurring secondary to a systemic disease because early initiation of treatment can improve clinical outcome, as manifested by our case.