The Successful Management of Diffuse Lymphangiomatosis Using Sirolimus: A Case Report

被引：53

作者：

Reinglas, Jason ^{[1
,2
]}

Ramphal, Raveena ^{[3
]}

Bromwich, Matthew ^{[1
]}

机构：

[1] Childrens Hosp Eastern Ontario, Div Otolaryngol Head & Neck Surg, Dept Otolaryngol, Ottawa, ON K1H 8L1, Canada

[2] Univ Ottawa, Fac Med, Ottawa, ON, Canada

[3] Childrens Hosp Eastern Ontario, Dept Pediat Hematol & Oncol, Ottawa, ON K1H 8L1, Canada

来源：

LARYNGOSCOPE | 2011年 / 121卷 / 09期

关键词：

Lymphangiomatosis; sirolimus; lymphatic malformations; airway; INTERFERON-ALPHA; KAPOSIFORM HEMANGIOENDOTHELIOMA; THORACIC LYMPHANGIOMATOSIS;

D O I：

10.1002/lary.21927

中图分类号：

R-3 [医学研究方法]; R3 [基础医学];

学科分类号：

1001 ;

摘要：

Lymphangiomatosis is a rare and fatal congenital lymphatic malformation. Because the natural course of the disease affects multiple body systems, the management can be challenging. This article presents a novel approach to the treatment of diffuse lymphangiomatosis using sirolimus. The reported case involves a 4-month-old male with a known lymphatic malformation who presented to the emergency department with respiratory difficulties. Sirolimus was successful at significantly reducing our patient's mass at a relatively low target level of 5 to 10 mu g/L. The use of sirolimus for the treatment of lymphangiomatosis should be studied further in the setting of a formal trial.

引用

页码：1851 / 1854

页数：4

共 20 条

[1] Thoracic lymphangiomatosis in a child [J].