Abstract
Purpose: A 56-year-old African-American man presented with a 1-week history of fatigue, nausea and vomiting, scleral icterus, light stools, and dark urine. He denied abdominal pain, fever, chills, or recent weight loss. Past medical history was significant for hypertension, asthma, and questionable lupus. He did not use alcohol, tobacco, or drugs, and had no recent travel events. His physical exam was relatively unremarkable aside from icterus. His white blood cell count was 51,000/mm3, with 82% eosinophils. Liver function tests were significant for an albumin of 2.4g/dL, total protein 8.7 g/dL, total bilirubin 15.3mg/dL, aspartate aminotransferase 248 IU/L, alanine aminotransferase 284 IU/L, alkaline phosphatase 845 IU/L, and lipase 322 IU/L. His INR was 1.35. CT of the abdomen showed a 2.4 cm x 1.5 cm enhancing lesion in the posterior segment of right hepatic lobe. Magnetic resonance cholangiopancreatography had non-visualization of a 1-cm segment of anterior branch of the right hepatic duct with mild dilation. A liver biopsy revealed marked portal mixed inflammation with numerous eosinophils, bile duct proliferation, and edematous fibrous stoma consistent with an inflammatory process and bile duct obstruction. No malignancy was seen. Endoscopic retrograde cholangiopancreatography revealed intra-hepatic duct beading with pruning, consistent with primary sclerosing cholangitis (PSC) or autoimmune cholangiopathy, with no extra-hepatic duct filling defects. Common bile duct brushings were negative for malignancy. A broad differential of infectious, allergic, and auto-immune causes were ruled out. Endoscopy did not reveal a GI malignancy. Bone marrow biopsy revealed hypercellular (˜70%) marrow with marked eosinophilia (˜36% of marrow cellularity), but no evidence of neoplastic causes. The patient remained stable throughout his hospitalization. He was discharged from the hospital with an indeterminate origin of hypereosinophilia. He was started on 60 mg oral prednisone daily, and had normalization of his symptoms and laboratory values within 2 months. This case illustrates the rare clinical entity of hypereosinophilia causing eosinophilic cholangiopathy. Clinical features were suggestive of a variety of disease processes, including primary sclerosing cholangitis (PSC), idiopathic hypereosinophilia syndrome (HES), and auto-immune cholangiopathy. Based upon literature review and further workup, this case likely represents a rare cholestatic manifestation of HES caused by eosinophilic infiltration of the biliary system. Treatment with steroids was both diagnostic and therapeutic.
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