HOMEPRODUCTSCOMPANYCONTACTFAQResearchDictionaryPharmaSign Up FREE or Login

Involvement of lymphatics in lymphangioleiomyomatosis.

Abstract
Lymphangioleiomyomatosis (LAM), a rare multisystem disease, occurs primarily in women, with cystic destruction of the lungs, abdominal tumors, and involvement of the axial lymphatics in the thorax and abdomen. To understand the pathogenesis of LAM, we initiated a longitudinal study of patients with LAM; over 500 patients have been enrolled. LAM results from the proliferation of a neoplastic cell (LAM cell), which has mutations in the tuberous sclerosis complex (TSC) genes, TSC1 or TSC2. Consistent with their metastatic behavior, LAM cells were isolated from blood, urine, and chylous effusions. Surface proteins on LAM cells include those found on metastatic cells and those involved in cell migration. In the lung, LAM cells are found clustered in nodules, which appear in the walls of the cysts, and in the interstitium. LAM lung nodules are traversed by slit-like vascular structures, with lining cells showing reactivity with antibodies against components of lymphatic endothelial cells. The axial lymphatics appear to be infiltrated by LAM cells, which may result in obstruction and formation of chyle-filled lymphangioleiomyomas. LAM cell clusters have been isolated from chylous pleural effusions, and it is hypothesized that these clusters may be responsible for metastatic spread of LAM cells via lymphatic vessels. Consistent with a lymphangiogenic process, levels of VEGF-D, a lymphangiogenic factor, were higher in sera of patients with LAM and lymphatic involvement (i.e., lymphangioleiomyoma, adenopathy) than in healthy volunteers or LAM patients with cystic disease limited to the lung. These findings are consistent with an important function for lymphangiogenesis in LAM.
AuthorsConnie G Glasgow, Angelo Taveira-DaSilva, Gustavo Pacheco-Rodriguez, Wendy K Steagall, Katsuya Tsukada, Xiong Cai, Souheil El-Chemaly, Joel Moss
JournalLymphatic research and biology (Lymphat Res Biol) Vol. 7 Issue 4 Pg. 221-8 (Dec 2009) ISSN: 1557-8585 [Electronic] United States
PMID20143921 (Publication Type: Comparative Study, Journal Article, Research Support, N.I.H., Intramural)
Chemical References
  • Vascular Endothelial Growth Factor D
Topics
  • Case-Control Studies
  • Female
  • Humans
  • Longitudinal Studies
  • Lymphangiogenesis (physiology)
  • Lymphangioleiomyomatosis (blood, etiology, pathology)
  • Lymphatic Vessels (physiology)
  • Male
  • Retrospective Studies
  • Vascular Endothelial Growth Factor D (blood)

Join CureHunter, for free Research Interface BASIC access!

Take advantage of free CureHunter research engine access to explore the best drug and treatment options for any disease. Find out why thousands of doctors, pharma researchers and patient activists around the world use CureHunter every day.
Realize the full power of the drug-disease research graph!


Choose Username:
Email:
Password:
Verify Password:
Enter Code Shown: