LYMPHANGIOLEIOMYOMATOSIS AND GORHAM-STOUT DISEASE: PRIMARY OR SECONDARY DISORDERS OF THE LYMPHATIC SYSTEM?

Abstract

Lymphangioleimyomatosis and Gorham-
Stout disease, rare disorders which share
features of proliferating lymphatic vessels,
predominantly in the lung in the former and
bone in the latter, commonly manifest as
progressive lung disease often associated with
life-threatening complications of chylous
reflux from central lymphatic obstruction/
leakage. This Lymphspiration-Editorial
proposes that, rather than a secondary complication,
lymphatic obstruction/ dysfunction is
a much earlier or even the primary event in
the pathogenesis of both disorders and that
lymphostasis drives the cellular proliferative
response in both lung and bone and even
distant sites.