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

Atsuko Nakatsuka; Jun Wada; Ryo Nagase; Masaya Takeda; Tadashi Yoshino; Hirofumi Makino

doi:10.2169/internalmedicine.46.6294

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

Atsuko Nakatsuka, Jun Wada, Ryo Nagase, Masaya Takeda, Tadashi Yoshino, Hirofumi Makino

Research output: Contribution to journal › Article › peer-review

6 Citations (Scopus)

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 language	English
Pages (from-to)	495-500
Number of pages	6
Journal	Internal Medicine
Volume	46
Issue number	8
DOIs	https://doi.org/10.2169/internalmedicine.46.6294
Publication status	Published - Apr 17 2007

Keywords

Hemophagocytic syndome
Liver failure
Macrophage
Veno-occlusive disease

ASJC Scopus subject areas

Internal Medicine

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10.2169/internalmedicine.46.6294

Cite this

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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 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.",

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T1 - Hemophagocytic syndrome associated with fatal veno-occlusive disease in the liver

AU - Nakatsuka, Atsuko

AU - Wada, Jun

AU - Nagase, Ryo

AU - Takeda, Masaya

AU - Yoshino, Tadashi

AU - Makino, Hirofumi

PY - 2007/4/17

Y1 - 2007/4/17

N2 - 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.

AB - 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.

KW - Hemophagocytic syndome

KW - Liver failure

KW - Macrophage

KW - Veno-occlusive disease

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

Abstract

Keywords

ASJC Scopus subject areas

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