2499 Idiopathic Eosinophilic Syndrome With Hepatic Involvement

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INTRODUCTION: Hepatic involvement in hypereosinophilic syndrome is rare. CASE DESCRIPTION/METHODS: A 66-year-old gentleman was referred for evaluation of persistent eosinophilia, cough, wheezing, and abnormal liver chemistries. No abdominal pain, fever, weight loss, and bowel habit changes. No traveling outside the U.S. On exam his vital signs were within normal range. Eyes, scleral icterus; Lungs; wheezing the bases; Abdomen, soft, nontender, nondistended, negative for, shifting dullness, no organomegaly, no distended veins on the abdominal wall; Extremities, no edema; Skin, no icterus no bruising. His initial laboratory findings were as follows, WBC 15.3 × 109/L, Hgb of 15.3 g/dL, platelets of 438 × 109/L, absolute neutrophil count of 6.7 × 109/L, absolute lymphocyte of 2.7 × 109/L, absolute eosinophil count of 4.5 × 109/L, alkaline phosphatase of 234 U/L, AST of 51 U/L, ALT of 94 U/L, total bilirubin of 0.4 mg/dL, albumin of 4.8 g/dL. With respect to the liver related laboratory findings: negative viral hepatitis panel, ANA of 1:160, total IgG of 1010 mg/dL, IgG1 of 659 mg/dL, IgG2 of 258 mg/dL, IgG3 of 12.9 mg/dL and IgG4 of 28 mg/dL; F-actin IgG antibody 23.3 units; negative anti-smooth muscle antibodies; soluble liver antibody <20 units; negative AMA, alpha-1 antitrypsin level of 167 mg/dL; and ceruloplasmin of 27 mg/dL. Comprehensive studies for stool ova and parasites was unremarkable. Peripheral blood smear showed eosinophilia. Bone marrow biopsy, with flow cytometric analysis revealing hypereosinophilia showing aberrant expression of CD 56. Abdominal MRI revealed a 5.8 cm hemangioma in the left lobe; no hepatic steatosis. Myeloproliferative neoplasms (MPN) and platelet-derived growth factor receptor α (PDGFRA A/B) workup was negative. Liver biopsy showed many eosinophils within portal areas with foci of sinusoidal involvement compatible with idiopathic eosinophilic syndrome; chronic hepatitis (grade 2/4), with portal and periportal fibrosis (stage II/IV); no steatosis. After starting prednisone 60 mg oral daily, tapered over 4 weeks, his cough, wheezing, hepatic enzymes' elevation and eosinophilia have resolved. DISCUSSION: Hypereosinophilic syndrome with hepatic involvement is rare, and should be in the differential diagnosis especially when hepatic enzymes' elevation is associated with eosinophilia.