S4355 Hypereosinophilic Syndrome—A Rare Cause of Hepatitis

Introduction: Hypereosinophilic syndrome (HES) is characterized by a serum eosinophil count of 1500/microlitre, 20% bone marrow involvement organ dysfunction due to tissue eosinophilia for 6 months without alternate cause. Diarrhea, fatigue and myalgia are common features. Hepatic, cerebral and cardiac involvement is rare. Diagnosis requires serum counts, tissue biopsy, bone marrow biopsy and genetic testing including PDGFRA gene. It also requires ruling out secondary causes parasitic infections, neoplasia or drug side effect. Case Description/Methods: Twenty-eight-year-old man presented with chest pain with elevated troponins. He had a negative left heart catheterization. It was non remarkable. On second day of hospitalization he had stroke like symptoms with subsequent computed tomography showing infarction in watershed frontal areas on the left side. He was started on aspirin, plavix and statin. Labwork revealed elevated AST, ALT, ALP and bilirubin. Serum eosinophils were 9000/microlitre. Granulomatosis with eosinophilia was suspected. ANCA, ANA and SSa/b was negative. There was no improvement with steroids. Patient was emperically started on Ivermectin the suspecting parasitic infection without improvement. Hepatitis panel including viral, autoimmune, Wilson's and Hemosiderosis were negative. Liver biopsy showed eosinophilia and bone marrow biopsy showed >20% eosinophilic cells. Serum FLP1-PDGFRA was checked and was positive revealing HES-myeloid variant. High dose steroids and then Imatinib was started with improvement in transaminases. A 5-year treatment was planned. Discussion: HES is multiorgan disorder requiring multidisciplinary approach depending on symptoms. Rheumatology, hematology, cardiology, gastroenterology and neurology were involved in this case. HES has 5 subtypes- Primary-HES- Myeloid, HES-Lymphoid, Secondary- like due to helminths, familial organ restricted and idiopathic. Diagnosis requires physician awareness and adequate work-up. Treatment includes high dose steroids, >1 g/day. Other first lines include hydroxyurea and then tyrosine kinase inhibitors like Imatinib. New trials for IL-5 antibodies are underway. Follow ups include monthly eosinophils count. Organ systems involved need to be followed like transaminases and troponins in outpatient.