Faculty Collaboration Database

A current viewpoint of lymphangioleiomyomatosis supporting immunotherapeutic treatment options. Am J Respir Cell Mol Biol 2012 Jan;46(1):1-5

Date

09/24/2011

Pubmed ID

21940815

DOI

10.1165/rcmb.2011-0215TR

Scopus ID

2-s2.0-84855365479 (requires institutional sign-in at Scopus site)   15 Citations

Abstract

Lymphangioleiomyomatosis (LAM) leads to hyperproliferation of abnormal smooth muscle cells in the lungs, associated with diffuse pulmonary parenchymal cyst formation and progressive dyspnea on exertion. The disease targets women of child-bearing age. Complications include pneumothoraces and chylous pleural effusions. Ten-year survival is estimated at 70%, and lung transplantation remains the only validated treatment. It has been observed that LAM cells express markers associated with melanocytic differentiation, including gp100 and MART-1. Other melanocytic markers have also been observed. The same proteins are targeted by T cells infiltrating melanoma tumors as well as by T cells infiltrating autoimmune vitiligo skin, and these antigens are regarded as relatively immunogenic. Consequently, vaccines have been developed for melanoma targeting these and other immunogenic melanocyte differentiation proteins. Preliminary data showing susceptibility of LAM cells to melanoma derived T cells suggest that vaccines targeting melanosomal antigens can be successful in treating LAM.

Author List

Dilling DF, Gilbert ER, Picken MM, Eby JM, Love RB, Le Poole IC

MESH terms used to index this publication - Major topics in bold

Biomarkers, Tumor
Humans
Immunotherapy
Lung Neoplasms
Lung Transplantation
Lymphangioleiomyomatosis