What is it about?
We examine the preliminary but promising findings which suggest that pulmonary lymphangioleiomyomatosis (LAM) could be treated by immunotherapy and suggest critical follow up studies.
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Why is it important?
LAM is a rare lung disease occurring almost exclusively in women that leads to progressive loss of lung function. The one clinically approved drug to treat LAM, rapamycin (sirolimus), only slows down the progression of the disease because the LAM cells are not killed by the drug. Therefore, new therapies are required to prolong the lives of LAM patients or potentially cure them. We examine important new studies in mice that suggest that LAM tumors evade the host immune system and that activation of the host immune system by immunotherapy improves the outcome of mouse models of LAM. Thus, additional studies are warranted to compare different types of immunotherapy in mouse models of LAM and the potential to combine rapamycin with immunotherapy. Finally, these findings suggest that immunotherapy should be explored for LAM patients who are non-responsive to rapamycin.
Perspectives
Many cancer therapies are based on killing rapidly dividing cells. Although LAM cells demonstrate some behavior reminiscent to cancer cells, they divide relatively slowly compared to cancer, and thus would not respond to most cancer therapeutics. In contrast, immunotherapy activates immune cells to kill cancer cells regardless of the growth rate of the cancer, suggesting that immunotherapy could be a potential treatment for LAM. Excitingly, the studies reviewed in our editorial support the notion that LAM could be targeted by immunotherapy.
William Stanford
Read the Original
This page is a summary of: Could Immunotherapy Sink its Teeth into LAM?, American Journal of Respiratory Cell and Molecular Biology, August 2018, American Thoracic Society,
DOI: 10.1165/rcmb.2018-0251ed.
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