A Discriminating Killer

摘要

One of tuberous sclerosis complex's (TSC's) most mysterious manifestations is lymphangi-oleiomyomatosis (LAM), a progressive lung disease that is decidedly sexist. The disease affects only women, usually in their childbearing years, and typically proves fatal within a decade or two of its diagnosis. Surprisingly, the tuberous growths that are a hallmark of TSC aren't the problem. Instead, LAM is caused by smooth muscle cells that invade the lungs. Researchers don't know where these invaders come from, nor what makes the cells spread to the lungs, where their accumulation slowly impairs breathing. "We don't know why the [cells] metastasize;... they look benign under the microscope," says Elisabeth Henske, an oncologist at Brigham and Women's Hospital in Boston who specializes in LAM. She notes that the disease can come back even after a patient has had a lung transplant, confirming that the source of the smooth muscle cells is somewhere other than the lungs.