title: Hemophagocytic syndrome associated with fatal veno-occlusive disease in the liver.
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 histiocytes 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-occlusive disease (VOD). Since VOD is regarded as a rare complication of HPS, the presence of VOD associated with HPS may be easily overlooked.
authors: Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine.
authors: Atsuko
authors: A
authors: Nakatsuka
authors: Jun
authors: J
authors: Wada
authors: Ryo
authors: R
authors: Nagase
authors: Masaya
authors: M
authors: Takeda
authors: Tadashi
authors: T
authors: Yoshino
authors: Hirofumi
authors: H
authors: Makino
mesh_terms: D017809:Fatal Outcome; D006504:Hepatic Veno-Occlusive Disease / Q000150:complications* / Q000175:diagnosis*; D006801:Humans; D017093:Liver Failure / Q000150:complications / Q000175:diagnosis; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications* / Q000175:diagnosis*; D008297:Male; D008875:Middle Aged