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成人斯蒂尔病相关噬血细胞综合征

Adult onset Still's disease associated hemophagocytosis.

作者信息

Kumakura S, Ishikura H, Munemasa S, Adachi T, Murakawa Y, Kobayashi S

机构信息

Third Division of Internal Medicine, Shimane Medical University, Izumo, Japan.

出版信息

J Rheumatol. 1997 Aug;24(8):1645-8.

PMID:9263166
Abstract

Reactive hemophagocytosis is characterized by the activation of histiocytes with prominent hemophagocytosis in the reticuloendothelial system, and usually occurs in association with underlying disorders such as viral or bacterial infection and malignancy. We describe 3 cases of adult onset Still's disease (AOSD) who developed hemophagocytosis in bone marrow. Extensive studies could not identify any viral infection or other known underlying disorders for reactive hemophagocytosis except for active AOSD. Although the causative mechanisms of reactive hemophagocytosis in AOSD are not clear, our cases suggest the possibility of AOSD associated hemophagocytosis.

摘要

反应性噬血细胞增多症的特征是组织细胞活化,伴有网状内皮系统中明显的噬血细胞现象,通常与潜在疾病如病毒或细菌感染及恶性肿瘤相关。我们描述了3例成人斯蒂尔病(AOSD)患者在骨髓中出现噬血细胞增多症的情况。广泛检查未发现除活动性AOSD外的任何病毒感染或其他已知的反应性噬血细胞增多症的潜在疾病。虽然AOSD中反应性噬血细胞增多症的致病机制尚不清楚,但我们的病例提示了AOSD相关噬血细胞增多症的可能性。

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