Erdheim Chester disease

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Erdheim-Chester Disease (pronounced: URD-hime CHES-ter disease) is a rare form of non-Langerhans cell histiocytosis.

Etymology

The disease is named after two pathologists, Jakob Erdheim from Austria and William Chester from the United States, who first described the condition in 1930.

Definition

Erdheim-Chester Disease is a rare type of non-Langerhans cell histiocytosis, characterized by the abnormal multiplication of a specific type of white blood cell known as histiocytes. These cells infiltrate various tissues and organs of the body, leading to inflammation and damage.

Symptoms

The symptoms of Erdheim-Chester Disease can vary greatly depending on the organs affected. Common symptoms include bone pain, neurological problems, cardiovascular issues, and renal impairment.

Diagnosis

Diagnosis of Erdheim-Chester Disease typically involves a combination of imaging studies, such as CT scans or MRI, and tissue biopsy.

Treatment

Treatment options for Erdheim-Chester Disease include chemotherapy, radiation therapy, and targeted therapies such as Vemurafenib and Cobimetinib.

Prognosis

The prognosis for Erdheim-Chester Disease is variable and depends on the extent of organ involvement and the patient's response to treatment.

Related Terms

External links

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