Generalized Lymphatic Anomaly

What is generalized lymphatic anomaly?

Generalized lymphatic anomaly (GLA) — also called lymphangiomatosis — is a rare condition involving the abnormal overgrowth of lymphatic vessels in the lungs, pleura (membranes around the lungs), bones and soft tissue.

Lymphatic vessels are part of the body’s lymphatic system, which transports a clear fluid containing white blood cells called lymph around the body to help clear toxins and waste.

The overgrowth of these vessels leads to lymphatic malformations, small or large cysts, formerly called lymphangiomas. Though benign (non-cancerous), these cysts can lead to serious problems.

There are many synonyms for GLA. Other names include:

  • diffuse pulmonary lymphangiomatosis, or DPL
  • pulmonary lymphangiomatosis

GLA is closely related to Gorham-Stout disease, a condition involving abnormal growth of lymphatic vessels that affects bone.

How we care for generalized lymphatic anomaly

At Boston Children’s Hospital, we treat patients with GLA in our Vascular Anomalies Center, considered a premier center in the world for vascular anomalies. Families of children around the globe with GLA seek care at Boston Children’s because we offer highly specialized care, access to leading clinical trials, a comprehensive multidisciplinary team approach and expert consults for national and international patients.

At Boston Children’s, our team is world renown for its expertise in vascular anomalies, including GLA. Our experts at the Vascular Anomalies Center are actively involved in the latest research on this disease, including participation in leading clinical trials.

Our areas of innovation for generalized lymphatic anomaly 

Boston Children’s is currently studying the use of immunosuppressant medications to relieve symptoms and slow the progression of GLA. Results have already shown significant success in patients.

These include:

  • Sirolimus (also known as rapamycin)
  • Interferon alfa-2b
  • VEGFR-3 inhibitors

We are also very active in the collection of patient data related to GLA to further improve our understanding of GLA, including what complications to expect, which therapies are more effective than others, predictors of disease stability and/or recurrence and inheritance patterns. Through this registry, we are studying causes of mortality, effusions, infections and other challenges in GLA. For more information or to participate, please visit our Lymphatic Anomalies Registry.