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一名16岁女孩患伴有瘀点疹的致命暴发性嗜酸性粒细胞增多综合征。

Fatal fulminant hypereosinophilic syndrome with a petechial rash in a 16-year-old girl.

作者信息

Scheinfeld Noah

机构信息

Saint Luke's-Roosevelt Hospital Center, Department of Dermatology, New York, NY 10028, USA.

出版信息

J Pediatr Hematol Oncol. 2003 Oct;25(10):827-30. doi: 10.1097/00043426-200310000-00020.

Abstract

Peripheral blood eosinophilia and eosinophilic tissue infiltration characterize the hypereosinophilic syndrome, which if untreated can be fatal. Its manifestations are protean. Often a diagnosis of exclusion, it presents diagnostic challenges. A fatal case of hypereosinophilic syndrome in a 16-year-old girl with petechiae, edema, urticaria, and diffuse erythema unfolded over 2 weeks. The histopathology of the petechiae demonstrated an eosinophilic and mixed cell interstitial and perivascular infiltrate and microthrombi in vessels. There was no evidence of T-cell clonality. Despite treatment with cyclosporine and methylprednisolone, she died. The age group; fulminant, fatal course without neoplasia; striking eosinophilia; and petechial rash are notable.

摘要

外周血嗜酸性粒细胞增多和嗜酸性组织浸润是高嗜酸性粒细胞综合征的特征,若不治疗可能会致命。其表现形式多样。通常是一种排除性诊断,存在诊断挑战。一名16岁女孩出现瘀点、水肿、荨麻疹和弥漫性红斑,在2周内病情进展为致命性高嗜酸性粒细胞综合征。瘀点的组织病理学显示嗜酸性粒细胞和混合细胞间质及血管周围浸润,血管内有微血栓形成。没有T细胞克隆性的证据。尽管使用环孢素和甲泼尼龙进行了治疗,她还是去世了。该年龄组;无肿瘤的暴发性、致命病程;显著的嗜酸性粒细胞增多;以及瘀点皮疹都值得关注。

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