ABSTRACT
Congenital hepatic fibrosis is a developmental abnormality that may appear either sporadically or in a familial form. It is an inherited disease defined pathologically by bands of fibrous tissue within the liver, and is occasionally associated with cystic kidney disease. A 21-year-old woman was admitted to our hospital for evaluation of pancytopenia. She showed esophageal varices, hepatomegaly and splenomegaly, but had normal results on her liver function test. Peripheral stigmata of chronic liver disease such as palmar erythema or spider angioma was not found. Hepatosplenomegaly, polycystic kidney and psoas muscle cyst were detected through an abdominal CT and MRI. The patient is diagnosis was confirmed as congenital hepatic fibrosis using laparoscopic liver biopsy. The first case of congenital hepatic fibrosis associated with polycystic kidney disease in Korea is herein reported.
Subject(s)
Female , Humans , Young Adult , Biopsy , Christianity , Diagnosis , Erythema , Esophageal and Gastric Varices , Fibrosis , Hemangioma , Hepatomegaly , Kidney Diseases, Cystic , Korea , Liver , Liver Diseases , Liver Function Tests , Magnetic Resonance Imaging , Pancytopenia , Polycystic Kidney Diseases , Psoas Muscles , Spiders , Splenomegaly , Tomography, X-Ray ComputedABSTRACT
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.
Subject(s)
Budd-Chiari Syndrome , Eosinophilia , Erythrocytes , Fibrosis , Hepatic Veins , Hypereosinophilic Syndrome , Liver , PathologyABSTRACT
Granulocytic sarcoma is localized myeloblastic cell tumor and has been described for many years in the literature as a rare manifestation of granulocytic leukemia. Although this tumor may involve anywhere in the body and give rise to a variety of signs and symptoms, there are several case reports of granulocytic sarcoma in Korea which described involvements at the lymph node, orbit, vagina, cervix, breast and mediastinum. We have experienced a case of granulocytic sarcoma only involving duodenum without hematologic evidence of leukemia and report this case with review of literature.