Onkológia 4/2024

Erdheim-Chester´s disease

Erdheim-Chester´s disease is an extremely rare dissease, that can affect many different organs of the body. It is an inflammatory hematologic disease, characterized by clonal proliferation, especially of foamy histiocytes and Touton giant cells. Typical is excessive accumulation of foam histiocytes , which infiltrate the loose connective tissue of the body. As a result this tissue becomes dense and fibrotic, multiple different organs can be affected and an organ failure can result. The most common presentation seen in over 90% of patients with ECD is the involvement of leg bones around the knees. The prognosis for ECD is variable and depends mainly on the extent and distribution of the disease. It ranges from asymptomatic bone lesions to multi-systemic life-threatening forms.

Keywords: histiocytes, Erdheim Chester's disease, diagnosis, clinical manifestations, treatment, clinical case