Internal medicine (Tokyo, Japan) · 2007
Hemophagocytic syndrome associated with fatal veno-occlusive disease in the liver.
Клинический случайСтатьяНазвание и аннотация: Перевод пока не подготовлен
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Nakatsuka Atsuko, Wada Jun, Nagase Ryo, Takeda Masaya, Yoshino Tadashi, Makino Hirofumi
Аннотация
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.