Fatal Idiopathic Hypereosinophilic Syndrome Presenting with Refractory Ascites : Case Report Study

Abstract

Hypereosinophilic syndromes were a group of divergent disorders united by overproduction of eosinophils and the several organ damages ascribed to this persistent eosinophilia. Among all the presenting symptoms, gastrointestinal symptoms were the least common. We were reporting a 21 year old man with a 2 year history of refractory ascites, hepatomegaly, portal and hepatic veins thrombosis and cutaneous lesions. Bone marrow aspiration and biopsy revealed granulocytic hyperplasia with marked eosinophilia. After ruling out common causes of eosinophilia, a diagnosis of idiopathic hypereosinophilic syndrome was made. The patient was treated with corticosteroids and imatinib but due to the advanced progression of the disease, resulted in a fatal outcome. Since early diagnosis and treatment is the key for improving the prognosis of HES patients, a high clinical suspicion is necessary in the diagnosis of this condition.

Keywords: Eosinophilia, Hypereosinophilic syndrome (HES), Refractory ascites