Generalized lymphatic anomaly successfully treated with long‐term,low‐dose sirolimus |
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Authors: | Veronika Dvorakova MUDr David Rea MCh Grainne M. O'Regan PhD Alan D. Irvine MD DSc |
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Affiliation: | 1. Department of Pediatric Dermatology, Our Lady's Children's Hospital, Crumlin, Dublin, Ireland;2. Department of Pediatric Radiology, Our Lady's Children's Hospital, Crumlin, Dublin, Ireland |
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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. |
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Keywords: | generalized lymphatic anomaly mTOR inhibitor sirolimus vascular malformation |
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