Learn About Erdheim-Chester Disease

What is the definition of Erdheim-Chester Disease?

Erdheim-Chester disease is a rare type of slow-growing blood cancer called a histiocytic neoplasm, which results in overproduction of cells called histiocytes. Histiocytes normally function to destroy foreign substances and protect the body from infection. In Erdheim-Chester disease, the excess production of histiocytes (histiocytosis) leads to inflammation that can damage organs and tissues throughout the body, causing them to become thickened, dense, and scarred (fibrotic); this tissue damage may lead to organ failure.

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What are the causes of Erdheim-Chester Disease?

More than half of people with Erdheim-Chester disease have a specific mutation in the BRAF gene. Mutations in other genes are also thought to be involved in this disorder.

How prevalent is Erdheim-Chester Disease?

Erdheim-Chester disease is a rare disorder; its exact prevalence is unknown. More than 500 affected individuals worldwide have been described in the medical literature. For unknown reasons, men are slightly more likely to develop the disease, accounting for about 60 percent of cases.

Is Erdheim-Chester Disease an inherited disorder?

This condition is not inherited. It arises from a somatic mutation in histiocytes or their precursor cells during an individual's lifetime.

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What are the latest Erdheim-Chester Disease Clinical Trials?
A Single-arm, Open Label, Multicenter Phase II Clinical Study in Rare Diseases to Evaluate Safety, Efficacy and PK of HLX208 for Adult Langerhans Cell Histiocytosis (LCH) and Erdheim-Chester Disease (ECD) With BRAF V600E Mutation
Summary: The purpose of this study was to assess safety, efficacy and PK in adult Langerhans Cell Histiocytosis (LCH) and Erdheim-Chester Disease (ECD) given HLX208 (BRAF V600E inhibitor).
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A Phase I Trial of Bevacizumab, Temsirolimus Alone and in Combination With Valproic Acid, or Cetuximab in Patients With Advanced Malignancy and Other Indications
Summary: This phase I trial studies the side effects and best dose of bevacizumab and temsirolimus alone or in combination with valproic acid or cetuximab in treating patients with a malignancy that has spread to other places in the body or other disease that is not cancerous. Immunotherapy with bevacizumab and cetuximab, may induce changes in body's immune system and may interfere with the ability of tumo...
Who are the sources who wrote this article ?

Published Date: April 01, 2017Published By: National Institutes of Health

What are the Latest Advances for Erdheim-Chester Disease?
Intermediate-dose cytarabine is an effective therapy for adults with non-Langerhans cell histiocytosis.
Summary: Intermediate-dose cytarabine is an effective therapy for adults with non-Langerhans cell histiocytosis.
Erdheim-Chester disease: a rare histiocytosis with outstanding response to cobimetinib.
Summary: Erdheim-Chester disease: a rare histiocytosis with outstanding response to cobimetinib.
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Granulomatous skeletal lesions.
Summary: Granulomatous skeletal lesions.