A rare case of association between Budd & #8211;Chiari syndrome and sea-blue histiocytosis

F Hu, Y Zhang, Z YiNigerian Journal of Clinical Practice 2019 22(11):1617-1620 Budd–Chiari syndrome (BCS) is a rare disease characterized by obstruction of hepatic venous outflow tract with diversified etiologies. Sea-blue histiocytosis (SBH) is a kind of storage diseases defined by the deposition of abundant sea-blue histiocytes in various organs and can lead to hepatosplenomegaly, cirrhosis, or even liver failure. The association between BCS and SBH has never been reported before. Here, we report a patient with BCS presenting with hepatosplenomegaly, portal hypertension, and pancytopenia who was later confirmed to also have SBH.
Source: Nigerian Journal of Clinical Practice - Category: Rural Health Authors: Source Type: research