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伴有血小板减少症的多灶性淋巴管内皮瘤病

Multifocal lymphangioendotheliomatosis with thrombocytopenia.

作者信息

Yeung Jensen, Somers Gino, Viero Sandra, Pope Elena

机构信息

Department of Dermatology, University of Toronto, Canada.

出版信息

J Am Acad Dermatol. 2006 May;54(5 Suppl):S214-7. doi: 10.1016/j.jaad.2005.07.029.

DOI:10.1016/j.jaad.2005.07.029
PMID:16631943
Abstract

Multifocal lymphangioendotheliomatosis with thrombocytopenia is an extremely rare disease. This condition manifests as diffuse congenital vascular lesions in the skin and gastrointestinal tract leading to severe gastrointestinal bleeding and thrombocytopenia. Histopathologic and immunohistochemical studies of vascular lesions demonstrate a lymphatic endothelial cell origin. Treatment often is not satisfactory. We herein describe a 4-week-old infant with this uncommon clinicopathologic entity.

摘要

伴有血小板减少的多灶性淋巴管内皮瘤病是一种极其罕见的疾病。这种病症表现为皮肤和胃肠道的弥漫性先天性血管病变,导致严重的胃肠道出血和血小板减少。对血管病变进行组织病理学和免疫组化研究显示其起源于淋巴管内皮细胞。治疗效果往往不尽人意。我们在此描述一名患有这种罕见临床病理实体的4周龄婴儿。

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