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The successful management of diffuse lymphangiomatosis using sirolimus: A case report

✍ Scribed by Jason Reinglas; Raveena Ramphal; Matthew Bromwich

Publisher: John Wiley and Sons
Year: 2011
Tongue: English
Weight: 252 KB
Volume: 121
Category: Article
ISSN: 0023-852X
DOI: 10.1002/lary.21927

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✦ Synopsis

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 lg/L. The use of sirolimus for the treatment of lymphangiomatosis should be studied further in the setting of a formal trial.

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