A Case of Secondary Sclerosing Cholangitis in the Setting of Non-Hodgkinʼs Lymphoma

Abstract

Introduction Sclerosing cholangitis represents a spectrum of cholestatic liver disease characterized by inflammation, fibrosis and stricture of the bile ducts. Magnetic resonance cholangiopancreatography (MRCP) typically shows multifocal strictures and dilatation of intra and extra-hepatic bile ducts displaying a “beaded” appearance. We present a case of secondary sclerosing cholangitis in the setting of anaplastic large cell lymphoma. Case Presentation A 67-year-old Caucasian female with a past history of recurrent breast cancer status post lumpectomy, radiation and hormonal therapy, presented with jaundice and an exophytic mass at the base of tongue. Laboratory data revealed ALP 953 U/L, total bilirubin 7.7 mg/dL, direct bilirubin 6.4 mg/dL, GGT 3369 U/L, albumin 3.1 g/dL, protein 6.9 g/dL, AST 195 U/L, ALT 149 U/L. A CT scan of the chest, abdomen and pelvis showed wide spread lymphadenopathy in the chest, abdomen and pelvis concerning for lymphoma, acute pancreatitis and biliary dilation with hyper enhancement of the common bile duct wall. An MRCP was performed which showed diffuse intrahepatic biliary ductal dilatation with non-visualization of the common hepatic duct bifurcation. Diffuse narrowing with multifocal stenosis of the proximal and distal aspects of the common bile duct was also seen. Findings were consistent with sclerosing cholangitis. Pathology of the oral lesion revealed ALK1+ anaplastic large cell lymphoma. Chemotherapy was initiated with cyclophosphamide, doxorubicin, adriamycin, vincristine, etoposide, and prednisone (CHOEP-14), and resulted in significant improvement in liver function tests. Patient continued to improve clinically and was discharged with close outpatient follow up. Discussion Previous reports have suggested that only 0.2 to 2.0 % of patients with NHL have biliary tract obstruction. The diagnosis of extra-hepatic bile duct obstruction related to lymphoma is challenging when it occurs as an initial presentation. The cholangiographic appearance of these lesions can resemble benign stricture, primary sclerosing cholangitis, cholangiocarcinoma, pancreatic carcinoma, or metastatic carcinoma. NHL as a secondary cause of sclerosing cholangitis has rarely been reported in literature. It is essential for gastroenterologists, oncologists and radiologists to recognize scelorsing cholangitis occurring secondary to systemic disease, as early initiation of treatment can improve clinical outcome, as happened in our case.Figure 1Figure 2