Budd-Chiari Syndrome Associated with Idiopathic Hypereosinophilic Syndrome / 대한혈액학회지

ABSTRACT

Idiopathic hypereosinophilic syndrome (IHS) can be diagnosed when prolonged eosinophilia and organ involvement are evident without specific etiologic factors. Budd-Chiari syndrome (BCS) is hepatic venous outflow obstruction, regardless of the cause or level of obstruction. Budd-Chiari syndrome associated with idiopathic hypereosinophic syndrome seems to be very rare, and only a few reports of such cases have been published. We experienced a 27-years-old man presented as BCS associated with idiopathic hypereosinophilic syndrome. Liver pathology revealed sinosoidal dilation, extravasated red blood cells and some degree of fibrosis. Liver sonogram, CT and inferior venocavogram didn't show visualization of hepatic vein and a short segmental narrowing of IVC. We present a case of BCS associated with IHS with review of literatures.