When Castleman Disease patients have a flare of their symptoms, they experience a cytokine storm inside their bodies—a hyper-response from the immune system that can cause a fever, organ failure, and even death. Now researchers at the Perelman School of Medicine say they know what’s happening at the cellular level of the immune system when these cytokine storms occur, and the answer not only informs future potential Castleman therapies but may also provide new insight into why similar events take place in COVID-19 patients. The researchers published their findings in the Journal of Clinical Investigation Insight.
Castleman Disease isn’t actually a single disease. The term describes a group of inflammatory disorders that share a common appearance under the microscope. It’s diagnosed in about 5,000 people of all ages each year in the United States, which makes it roughly as common as Lou Gehrig’s disease, also called Amyotrophic Lateral Sclerosis or ALS. Patients experience a range of symptoms—from a single abnormal lymph node with mild flu-like symptoms to abnormal lymph nodes located throughout their entire body, abnormal blood cell counts, and life-threatening failure of multiple organ systems, such as the kidneys, liver, heart, and lungs. The most severe subtype, idiopathic multicentric Castleman disease (iMCD), has similarities to autoimmune conditions, viral illnesses, and cancer. About 35 percent of patients with iMCD will die within five years of diagnosis.
“With iMCD, just as with COVID-19, it is the body’s hyper-response that’s deadly rather than the disease itself, and this study gives us new clues about why the immune cells are out of control and what we can do to rein them in,” says the study’s senior author David C. Fajgenbaum, an assistant professor of translational medicine and human genetics, director of the Center for Study & Treatment of Lymphadenopathies & Cytokine Storms, executive director of the Castleman Disease Collaborative Network, and a Castleman patient himself.
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