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噬血细胞增多症并发川崎病。

Hemophagocytosis complicating Kawasaki disease.

作者信息

al-Eid W, al-Jefri A, Bahabri S, al-Mayouf S

机构信息

Department of Pediatrics, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.

出版信息

Pediatr Hematol Oncol. 2000 Jun;17(4):323-9. doi: 10.1080/088800100276316.

Abstract

A 6-year-old boy developed hemophagocytic syndrome during the recurrent course of Kawasaki disease. Despite the appropriate treatment modalities for Kawasaki disease, he developed pancytopenia, marked hepatosplenomegaly, high-grade fever, hyperferritinemia, hypertriglyceremia, and evidence of hemophagocytosis in the liver biopsy. Although the course was stormy, he responded well to a combination therapy of corticosteroids, etoposide VP16, and granulocyte colony-stimulating factor G-CSF. The clinical course and the treatment given were compared with the previous reported cases.

摘要

一名6岁男孩在川崎病复发过程中出现噬血细胞综合征。尽管采用了针对川崎病的适当治疗方式,但他仍出现了全血细胞减少、明显的肝脾肿大、高热、高铁蛋白血症、高甘油三酯血症,并且肝活检显示有噬血细胞现象。尽管病程凶险,但他对皮质类固醇、依托泊苷(VP16)和粒细胞集落刺激因子(G-CSF)的联合治疗反应良好。将该临床病程及所给予的治疗与先前报道的病例进行了比较。

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