A rare case of association between Budd-Chiari syndrome and sea-blue histiocytosis

Niger J Clin Pract. 2019 Nov;22(11):1617-1620. doi: 10.4103/njcp.njcp_43_19.


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.

Keywords: Bone marrow aspiration and biopsy; Budd–Chiari syndrome; hepatosplenomegaly; portal hypertension; sea-blue histiocytosis.

Publication types

  • Case Reports

MeSH terms

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