Generalized lymphatic anomaly successfully treated with long‐term, low‐dose sirolimus. Issue 4 (26th March 2018)

• Record Type: Journal Article
• Authors: Dvorakova, Veronika; Rea, David; O'Regan, Grainne M.; Irvine, Alan D.
• Is Part Of: Pediatric dermatology. Volume 35:Issue 4(2018)
• Page Start: 533
• Page End: 534
• Publication Date: 2018-03-26
• Subjects: generalized lymphatic anomaly -- mTOR inhibitor -- sirolimus -- vascular malformation; Pediatric dermatology -- Periodicals; Children -- Diseases -- Periodicals; 618.925
• DOI: 10.1111/pde.13494
• Languages: English
• ISSNs: 0736-8046

Abstract

Abstract: 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.