Hemophagocytic syndrome associated with fatal veno-occlusive disease in the liver

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Abstract

A 47-year-old man presented with hemophagocytic syndrome (HPS) without any obvious underlying diseases. On computed tomography, his liver was occupied by multiple ill-defined low intensity lesions. Liver biopsy revealed diffuse infiltration of numerous histiocytes without cytologic atypism and prominent fibrotic changes. These histiocyes showed S100(+), CD68(+), CD1a(-), and lysozyme(+) and Langerhans cell granules were not observed by electron microscopic examination. He failed to respond to immunosuppressive and chemotherapeutic treatments and progressed to severe liver failure. At autopsy, his liver exhibited veno-occulusive disease (VOD). Since VOD is regafded as a rare complication of HPS, the presence of VOD associated with HPS may be easily overlooked.

Original languageEnglish
Pages (from-to)495-500
Number of pages6
JournalInternal Medicine
Volume46
Issue number8
DOIs
Publication statusPublished - Apr 17 2007

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Keywords

  • Hemophagocytic syndome
  • Liver failure
  • Macrophage
  • Veno-occlusive disease

ASJC Scopus subject areas

  • Internal Medicine

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