Hepatitis in Idiopathic Hypereosinophilic Syndrome: Report of an Unusual Case

Abstract

World Health Organization defines a rare diagnosis Idiopathic hypereosinophilic syndrome (HES) as a persistent eosinophilia for 6 months and resulting in end-organ dysfunction. Most of the patients present with nonspecific symptoms, while others will present with symptoms of the affected organs, commonly those involving the heart, skin, or nervous system. Gastrointestinal or liver involvement is estimated to affect up to one-third of patients with HES, although patients with clinically significant disease are limited to case reports. This is the first report of a patient presenting with idiopathic HES related hepatitis and achalasia. Hypereosinophilic syndrome has been reported to be associated with hepatic dysfunction; liver histology is mainly characterized by a diffuse eosinophilic inflammatory infiltrate. A 49-yr-old woman, diagnosed as a case of idiopathic hypereosinophilic syndrome with bone marrow and pulmonary eosinophilic infiltrates associated with peripheral eosinophilia,high IgE level developed features of chronic gatrornteritis, hepatitis, with a significant eosinophil component. She responded well to systemic glucocorticoid and Imatinib therapy with normalization of liver function tests within a few weeks.