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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.

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.

Who are the top Erdheim-Chester Disease Local Doctors?
Elite in Erdheim-Chester Disease
Elite in Erdheim-Chester Disease

Memorial Neurology Group

160 E 53rd St, 
New York, NY 
Languages Spoken:
English
Offers Telehealth

Eli Diamond is a Neurologist in New York, New York. Dr. Diamond is rated as an Elite provider by MediFind in the treatment of Erdheim-Chester Disease. His top areas of expertise are Erdheim-Chester Disease, Glioblastoma, Astrocytoma, Brain Tumor, and Awake Craniotomy.

Jithma P. Abeykoon
Elite in Erdheim-Chester Disease
Hematology | Oncology | Hematology Oncology
Elite in Erdheim-Chester Disease
Hematology | Oncology | Hematology Oncology

Mayo Clinic

200 1st St Sw, 
Rochester, MN 
Languages Spoken:
English
Accepting New Patients

Jithma Abeykoon is a Hematologist and an Oncologist in Rochester, Minnesota. Dr. Abeykoon is rated as an Elite provider by MediFind in the treatment of Erdheim-Chester Disease. His top areas of expertise are Waldenstrom Macroglobulinemia, Histiocytosis, Reticulohistiocytoma, Tissue Biopsy, and Bone Marrow Transplant. Dr. Abeykoon is currently accepting new patients.

 
 
 
 
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Elite in Erdheim-Chester Disease
Elite in Erdheim-Chester Disease
Paris, FR 

J Haroche practices in Paris, France. Haroche is rated as an Elite expert by MediFind in the treatment of Erdheim-Chester Disease. Their top areas of expertise are Reticulohistiocytoma, Histiocytosis, Erdheim-Chester Disease, Non-Langerhans-Cell Histiocytosis, and Tissue Biopsy.

What are the latest Erdheim-Chester Disease Clinical Trials?
DETERMINE (Determining Extended Therapeutic Indications for Existing Drugs in Rare Molecularly Defined Indications Using a National Evaluation Platform Trial): An Umbrella-Basket Platform Trial to Evaluate the Efficacy of Targeted Therapies in Rare Adult, Paediatric and Teenage/Young Adult (TYA) Cancers With Actionable Genomic Alterations, Including Common Cancers With Rare Actionable Alterations. Treatment Arm 05: Vemurafenib in Combination With Cobimetinib in Adult Patients With BRAF Positive Cancers.

Summary: This clinical trial is looking at a combination of drugs called vemurafenib and cobimetinib. Vemurafenib is approved as standard of care for adult patients with unresectable or metastatic melanoma. Cobimetinib is approved as standard of care in combination with vemurafenib for the treatment of adult patients with unresectable or metastatic melanoma. This means it has gone through clinical trials a...

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MC220806: Phase I Study Evaluating the Efficacy of CSF1R and TAM Receptor or Inhibition in Hematologic Malignancies With Q702, a Small Molecular Inhibitor

Summary: This phase I trial tests the safety, side effects, and best dose of Q702 in treating patients with hematologic malignancies. Q702 is in a class of medications called immunomodulatory agents. It works by helping the immune system kill cancer cells and by helping the bone marrow to produce normal blood cells. Giving Q702 may be safe, tolerable and/or effective in treating patients with hematologic m...

Who are the sources who wrote this article ?

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