Erdheim Chester disease
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
- Histiocytosis
- Non-Langerhans cell histiocytosis
- Langerhans cell histiocytosis
- Vemurafenib
- Cobimetinib
External links
- Medical encyclopedia article on Erdheim Chester disease
- Wikipedia's article - Erdheim Chester disease
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