Researchers Discover New Insights About Lymphangioleiomyomatosis

Scientists have found new insights about lymphangioleiomyomatosis (LAM), a rare lung disease, which affects approximately 1 in 200,00 Americans and typically includes the growth of abnormal cells in different tissues and organs, including the lungs.

Lymphangioleiomyomatosis research

As scientists studied LAM cells in the lab, they found a “mixed phenotype,” or distinctions in physical expression in the lymphatic endothelial cells. These cells seemed to look like cells in lymphatics in addition to blood endothelial cells.

The attributes were also observed in a small percentage of lung cells supplied by individuals living with idiopathic pulmonary fibrosis (IPF), a different rare lung disease. However, the scientists did not observe this phenotypical crossover when examining cells from healthy volunteers or from individuals suffering from Kaposi’s sarcoma, a blood-related cancer.

When LAM cells build up in the lungs, cysts can create and impede air flow, making breathing challenging. To assist people coping with LAM and other rare lung conditions, scientists have actually been examining the physical attributes of LAM.

LAM cells in the lung reside in nodules, which are small clusters of LAM and other cells that line the cysts and might be dispersed. These lung nodules also have lymphatics, part of the lymphatic system, sustaining circulation and immune function. Lymphatic endothelial cells line the nodules and offer new insight regarding LAM.

Scientists anticipate that continuous study and future research will lead to customized therapies for individuals living with LAM and other rare lung conditions. They also recommend researching and will continue to comply with the role lymphatics may have in disease progression, which might sustain the development of new and improved therapies.

Read the original article on News Medical.

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Reference: Pacheco-Rodriguez, G., et al. (2022) A mixed blood-lymphatic endothelial cell phenotype in LAM and IPF but not in Kaposi’s sarcoma or TSC. American Journal of Respiratory Cell and Molecular Biology. doi.org/10.1165/rcmb.2021-0293LE.