Chronic Hepatitis E in a Case of Chronic Lymphocytic Leukemia: A Rare Presentation of Chronic HEV in a Non-transplant Patient

Abstract

A 72-year-old French man with chronic lymphocytic leukemia (CLL) evaluated for continued unexplained jaundice, elevated transaminases, and fatigue for five months. His initial labs were remarkable for elevated transaminases [AST 134 U/L, ALT 216 U/L] and mild hyperbilirubinemia [1.4 mg/dL]. A liver biopsy revealed chronic hepatitis with mild portal fibrosis, focal mild periportal fibrosis, and steatosis. The pattern of lymphocytic staining was not compatible with CLL, and immunochemistry was negative for CLL. There were no intracytoplasmic inclusions, negative for alpha 1 AT deficiency, no Mallory bodies, and negative for Prussian blue stain. His past medical history was most significant for CLL first diagnosed 10 years prior to the initial hepatology visit. Rituxan based chemotherapy was started 4 years ago. Medical history is also notable for coronary artery disease s/p PCI with stents at age 68. He had an appendectomy during childhood and a cholecystectomy at 67 years old. He consumes one glass of wine every other day and has a former 50 pack year smoking history. He endorsed occasional consumption of undercooked pork and beef tartare every couple months, which is a delicacy in his home in Marseilles, France. His last visit to Southwestern France was 6 years ago, and his last foreign travel was on a cruise trip to Central America 1 years ago. There was no clinically apparent hepatomegaly or splenomegaly. An initial laboratory work-up was unrevealing, including hepatitis A, B and C serologies & viral count, ASMA, AMA, ANA, ferritin, and ceruloplasmin. However, Hepatitis E Ab Ig M was detected positive on the initial hepatology clinic visit. The test was repeated and positive again one month later. Samples sent for HEV RNA detection at the CDC lab was positive. The patient was confirmed to have Chronic Hepatitis E and started on ribavirin 600mg twice daily for 3 months. Previously known as a self-limiting acute infection, hepatitis E virus (HEV) has been found to cause chronic hepatitis, particularly in post organ transplant patients from Southwestern France on immunosuppressive therapy and especially in those consuming undercooked pork. While awareness of chronic Hepatitis E is growing, it is not commonly reported outside of post-transplant recipients. Chronic hepatitis E should be considered in the differential for all immunosuppressed patients with similar epidemiological risk factors and unexplained chronic hepatocellular disease.