1410 A Rare Case of Extrahepatic Cholestasis Secondary to Acquired Immunodeficiency Syndrome (AIDS)—Related Kaposi Sarcoma

Abstract

INTRODUCTION: Kaposi sarcoma is an aggressive cancer seen in immunocompromised patients caused by Human Herpesvirus-8 (HHV-8) infection. Cutaneous and pulmonary complications are the most frequent, biliary tract involvement is rarely reported, typically asymptomatic but may present as cholangitis or progress to liver failure. CASE DESCRIPTION/METHODS: 35-year-old male with HIV and CD4 count of 35 cells/uL, not on antiretroviral therapy for the past year, presented with fever, fatigue, diffuse lymphadenopathies, unintentional weight loss, generalized abdominal pain and scleral icterus for the last 2 weeks. Physical exam showed icterus, small hyperpigmented lesions throughout his trunk, a violaceous nodular enlargement of the hard palate, generalized lymphadenopathy, diffusely tender abdomen and hepatomegaly. Laboratory studies were significant for total bilirubin 23 mg/dL, direct bilirubin 16 mg/dL, ALP 288 U/L, GGT 193 U/L, AST 66 U/L, ALT 40 U/L, INR 1.3. CT of the abdomen showed hepatomegaly and an infiltrative process in the portal hepatis to the celiac area with obstruction of the biliary tract with intrahepatic biliary dilation. Endoscopic retrograde cholangiopancreatography demonstrated 3 cm stricture in the common hepatic duct, a biopsy was taken, and 2 stents were placed. Endoscopic ultrasound with biopsies taken from a stomach nodule and porta hepatis were performed as well. Pathology from porta hepatis mass showed blood clot however, HHV-8 stain from the common hepatic duct and stomach nodule biopsies was positive for Kaposi sarcoma. Doxorubicin and antiretroviral therapy were started, and patient was discharged in view of symptomatic improvement. Liver function tests normalized 8 weeks after presentation. DISCUSSION: Kaposi sarcoma involving the biliary tract has been associated with cholangitis and jaundice and may also be caused from extensive disease of the porta hepatis with compression of the extrahepatic biliary tree. Current first-line treatment for advanced Kaposi sarcoma is lipososomal anthracyclines (doxorubicin, daunorubicin). HHV-8 infection cannot be eradicated, but long-term remission is possible. Treatment is indicated in patients with progressive hepatic and biliary disease. Kaposi sarcoma has also been associated with AIDS (acquired immunodeficiency syndrome) cholangiopathy, a form of biliary tract inflammation with stricture formation in severely immunosuppressed HIV patients and management consists of endoscopic sphincterotomy for papillary stenosis and stenting.