关键词: Bone marrow aspiration and biopsy Budd–Chiari syndrome hepatosplenomegaly portal hypertension sea-blue histiocytosis

Mesh : Adult Budd-Chiari Syndrome / complications diagnosis etiology Hepatomegaly / complications diagnostic imaging Humans Hypertension, Portal / complications Male Pancytopenia / complications Rare Diseases Sea-Blue Histiocyte Syndrome / diagnosis Splenomegaly / complications diagnostic imaging Vena Cava, Inferior

来  源:   DOI:10.4103/njcp.njcp_43_19

Abstract:
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.
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