European Respiratory Review (Jun 2016)

Thoracic involvement in generalised lymphatic anomaly (or lymphangiomatosis)

  • Francesca Luisi,
  • Olga Torre,
  • Sergio Harari

DOI
https://doi.org/10.1183/16000617.0018-2016
Journal volume & issue
Vol. 25, no. 140
pp. 170 – 177

Abstract

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Generalised lymphatic anomaly (GLA), also known as lymphangiomatosis, is a rare disease caused by congenital abnormalities of lymphatic development. It usually presents in childhood but can also be diagnosed in adults. GLA encompasses a wide spectrum of clinical manifestations ranging from single-organ involvement to generalised disease. Given the rarity of the disease, most of the information regarding it comes from case reports. To date, no clinical trials concerning treatment are available. This review focuses on thoracic GLA and summarises possible diagnostic and therapeutic approaches.