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无血小板减少或系统性出血临床体征的多灶性淋巴管内皮瘤病

Multifocal lymphangioendotheliomatosis without thrombocytopenia or clinical signs of systemic bleeding.

作者信息

Peña Merino Lander, López Almaraz Ricardo, Fernández de Larrinoa Aitor, Rubio Lombraña Marta, González-Hermosa Maria Rosario

机构信息

Department of Dermatology, Hospital Universitario Cruces, Barakaldo, Spain.

Department of Pediatric Oncology, Hospital Universitario Cruces, Barakaldo, Spain.

出版信息

Pediatr Dermatol. 2019 Nov;36(6):965-966. doi: 10.1111/pde.13959. Epub 2019 Aug 25.

Abstract

Multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT) is an extremely rare recently described disorder characterized by diffuse congenital skin and gastrointestinal vascular lesions that may be associated with gastrointestinal bleeding and thrombocytopenia. We herein present a case report of multifocal lymphangioendotheliomatosis without thrombocytopenia or extensive extracutaneous involvement (gastrointestinal bleeding). Given the high morbidity and mortality associated with this disease, it is important for clinicians to recognize this disorder in order to select the most appropriate therapeutic approach.

摘要

多灶性淋巴管内皮瘤病伴血小板减少症(MLT)是一种最近才被描述的极为罕见的疾病,其特征为先天性弥漫性皮肤和胃肠道血管病变,可能伴有胃肠道出血和血小板减少症。我们在此报告一例无血小板减少症或广泛皮肤外受累(胃肠道出血)的多灶性淋巴管内皮瘤病病例。鉴于该疾病相关的高发病率和死亡率,临床医生认识这种疾病以便选择最合适的治疗方法非常重要。

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