Generalized lymphatic anomaly successfully treated with long‐term, low‐dose sirolimus

Generalized lymphatic anomaly is a rare, complex, lymphatic anomaly generally involving soft tissues, spleen, and bones. It can lead to focal skeletal fragility and pathologic effusions. A recent prospective trial of sirolimus for complicated vascular anomalies showed partial response in seven patie...

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Bibliographic Details
Published in:Pediatric dermatology 2018-07, Vol.35 (4), p.533-534
Main Authors: Dvorakova, Veronika, Rea, David, O'Regan, Grainne M., Irvine, Alan D.
Format: Article
Language:English
Subjects:
Bone strength
Case reports
generalized lymphatic anomaly
mTOR inhibitor
Rapamycin
sirolimus
Soft tissues
Spleen
vascular malformation
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Summary:Generalized lymphatic anomaly is a rare, complex, lymphatic anomaly generally involving soft tissues, spleen, and bones. It can lead to focal skeletal fragility and pathologic effusions. A recent prospective trial of sirolimus for complicated vascular anomalies showed partial response in seven patients with generalized lymphatic anomaly treated with sirolimus with a target trough level of 10‐15 ng/mL for 1 year (Adams et al). We describe successful treatment of generalized lymphatic anomaly with a lower‐dose, long‐term course of sirolimus.
ISSN:0736-8046
1525-1470
DOI:10.1111/pde.13494