A Case of Hepatoportal Sclerosis in a Patient Receiving Thioguanine Treatment for ALL

Abstract

Introduction: Hepatoportal sclerosis is a known cause of noncirrhotic portal hypertension. We present the case of a patient with ALL on active chemotherapy with mercaptopurine who presented with upper GI bleeding in the setting of portal hypertension and was later discovered to have hepatoportal sclerosis on liver biopsy. Case: A 20 year old male was seen in the adult GI clinic for management of newly diagnosed hepatosportal sclerosis. He had a history of ALL diagnosed at the age of nine and was started on intrathecal methotrexate, 6-mercaptopurine, vincristine, doxorubicin, and prednisolone. He was treated for approximately three years after which he went into remission. Prior to completing his chemotherapy, he developed splenomegaly and thrombocytopenia. This was initially attributed to chemotherapy and was monitored closely. Five years later, the patient was admitted to the hospital with hematemesis and bloody stools. An EGD showed grade I and II esophageal varices for which he underwent banding and a liver biopsy showed findings of hepatoportal sclerosis. A CT abdomen showed patent portal, renal, and hepatic veins and a repeat transjugular liver biopsy a few years later revealed findings of nodular regenerative hyperplasia. The patient tested negative for hepatitis A,B,C, parasitic diseases, and HIV and an autoimmune workup was negative for rheumatologic diseases, primary biliary cirrhosis, and autoimmune hepatitis. Discussion: There has been only one case report of an association of 6-mercaptopurine and hepatoportal sclerosis. We present a second case where the cause of hepatoportal sclerosis was attributed to mercaptopurine. Clinicians should be aware of mercaptopurine's short and long-term side effects in order to implement appropriate treatment early and prevent long-term complications