A current viewpoint of lymphangioleiomyomatosis supporting immunotherapeutic treatment options Journal Article

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Authors: Dilling, D. F.; Gilbert, E. R.; Picken, M. M.; Eby, J. M.; Love, R. B.; Le Poole, I. C.
Article Title: A current viewpoint of lymphangioleiomyomatosis supporting immunotherapeutic treatment options
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.
Journal Title: American Journal of Respiratory Cell Molecular Biology
Volume: 46
Issue: 1
ISSN: 1535-4989
Publisher: Unknown  
Journal Place: United States
Date Published: 2012
Start Page: 1
End Page: 5
Language: English
DOI/URL:

http://ovidsp.ovid.com/ovidweb.cgi?T=JS=Y=N=fulltext=medl=21940815
Notes: ID: 12322; Record Owner: From MEDLINE, a database of the U.S. National Library of Medicine.; Status: MEDLINE; [Review]; Publishing Model: Journal available in: Print Citation processed from: Internet; NLM Journal Code: aob, 8917225; CAS Registry/EC Number/Name of Substance: 0 (Tumor Markers, Biological).; Entry Date: 20120503