What is Erdheim-Chester Disease?

Erdheim-Chester Disease (ECD) is an extremely rare blood cancer that can affect many different organs of the body. It is characterized by excessive production and accumulation of specific cells whose normal functions are to fight infections and eliminate foreign bodies. These cells, called histiocytes, infiltrate the loose connective tissue within body organs and cause inflammation. As a result, this tissue becomes thickened, dense, and fibrotic. If the disease is not controlled, organ failure can occur. In some patients, ECD may have a relatively benign course involving a limited number of organs. In contrast, in others, the disease can be more aggressive and may affect multiple organs, including, but not limited to, the bones, skin, heart, kidney, and brain.

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