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[1例表现为成人斯蒂尔病的噬血细胞综合征合并严重肝损伤]

[A case of hemophagocytic syndrome with severe liver injury manifestating adult Still's disease].

作者信息

Ishikawa M, Masumoto T, Oguni T, Hyoudou N, Michitaka K, Horiike N, Onji M

机构信息

Third Department of Internal Medicine, Ehime University School of Medicine Tushima Public Hospital.

出版信息

Nihon Rinsho Meneki Gakkai Kaishi. 1997 Oct;20(5):447-52. doi: 10.2177/jsci.20.447.

Abstract

In April 1988, a 23 year-old woman developed high fever, arthralgia, eruptions and splenomegaly. She was treated with non Steroid anti-inflammatory drugs, and the symptoms disappeared. In June 1991, she was diagnosed as adult Still's disease and treated with prednisolone. In July 1994, she was treated with pulse therapy methylprednisolone due to high fever, eruptions, arthralgia and the high levels of ferritin. However, due to the marked increase of serum transaminase and bilirubin levels, she was referred to University hospital. She developed hepatic failure after admission Bone-marrow puncture revealed hemophagocytosis. She died ten days after admission. She was diagnosed as hemophagocytic syndrome combined with acute hepatic failure.

摘要

1988年4月,一名23岁女性出现高热、关节痛、皮疹和脾肿大。她接受了非甾体抗炎药治疗,症状消失。1991年6月,她被诊断为成人斯蒂尔病,并接受泼尼松龙治疗。1994年7月,由于高热、皮疹、关节痛和铁蛋白水平升高,她接受了甲泼尼龙冲击治疗。然而,由于血清转氨酶和胆红素水平显著升高,她被转诊至大学医院。入院后她出现肝功能衰竭。骨髓穿刺显示噬血细胞现象。入院十天后她死亡。她被诊断为噬血细胞综合征合并急性肝功能衰竭。

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