Idiopathic hypereosinophilic syndrome with gastrointestinal involvement

Abstract

Idiopathic hypereosinophilic syndrome (iHES) is a rare disorder with an unclear cause that is characterized by a persistent increase in the number of eosinophilic granulocytes in the peripheral blood and organ damage. The differential dia­gnosis of hypereosinophilia is broad – the cause may be primary (clonal), secondary (reactive – most often in the context of allergic, parasitic, autoimmune or malignant diseases), genetic (familial) or idiopathic (determined per exclusionem). This case report describes a patient with recurrent episodes of abdominal pain, ascites, and leukocytosis with hypereosinophilia, who underwent extensive examinations over the years, including two dia­gnostic laparoscopies. CT scans revealed abnormalities in the small bowel and stomach region, and histological and cytological examinations confirmed an increased number of eosinophils in the duodenal villi and eosinophilic ascites. Gastroscopy raised suspicion of eosinophilic esophagitis and stomach involvement, but this was not confirmed histologically. The ethiology of mild hepatopathy with elevated transaminases remains unclear, as the patient refused liver bio­psy, repeatedly. After ruling out primary and secondary causes of hypereosinophilia, the idiopathic hypereosinophilic syndrome was dia­gnosed and corticotherapy was initiated with good clinical and laboratory outcome and normalization of eosinophil count.