Erdheim Chester can be life-threatening with complications such as heart failure, severe damage to the lungs, and kidney failure. However, with treatment, there are patients who are able to live a near-normal life.
Moreover, how common 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.
Similarly one may ask, is erdheim Chester disease an autoimmune disease?
Erdheim-Chester disease (ECD) is an infrequent, autoimmune disorder that is not Langerhans histiocytosis and is characterized by bilateral sclerosis of the diametaphyseal medullary regions of the long bones and possible multiorgan involvement.
What causes histiocytosis?
The exact cause of histiocytosis is unknown. However, recent studies indicate that it is caused by the development and expansion of an abnormal Langerhans cell that subsequently leads to the accumulation of other cells of the immune system, resulting in collections or tumors in various areas of the body.
What does a histiocyte do?
A histiocyte is a type of immune cell. It destroys foreign substances to protect the body from infection.
What is erdheim Chester syndrome?
Erdheim-Chester disease (ECD) is a rare multisystem disorder of adulthood. It is characterized by excessive production and accumulation of histiocytes within multiple tissues and organs. Histiocytes are large phagocytic cells (macrophages) that normally play a role in responding to infection and injury.
What is Langerhans Cell Histiocytosis?
Langerhans cell histiocytosis is a rare disorder that can damage tissue or cause lesions to form in one or more places in the body. Langerhans cell histiocytosis (LCH) is a rare disease that begins in LCH cells. LCH cells are a type of dendritic cell that normally helps the body fight infection.
What is the rarest disease in the world?
Five rare diseases you never knew existed
- Stoneman Syndrome. Frequency: one in two million people. …
- Alice In Wonderland Syndrome (AIWS) Frequency: currently unknown. …
- Hutchinson-Gilford Progeria Syndrome (HGPS) Frequency: one in four million. …
- Alkaptonuria. …
- Chronic Focal Encephalitis (Rasmussen’s Encephalitis)
What is the treatment for erdheim Chester?
The U.S. Food and Drug Administration today expanded the approval of Zelboraf (vemurafenib) to include the treatment of certain adult patients with Erdheim-Chester Disease (ECD), a rare cancer of the blood. Zelboraf is indicated to treat patients whose cancer cells have a specific genetic mutation known as BRAF V600.
When was erdheim-Chester disease discovered?
Erdheim-Chester Disease (ECD) is a rare form of non Langerhans’ cell histiocytosis originally described as “Lipid Granulomatosis” in 1930 by Jakob Erdheim and William Chester.
WHO classification erdheim-Chester disease?
The revised 2016 World Health Organization classification introduced Erdheim-Chester disease (ECD) as a provisional entity within the histiocytic and dendritic cell neoplasms separate from the juvenile xanthogranuloma family based on distinct molecular features.
Why macrophages are called histiocytes?
Macrophages and dendritic cells are derived from common bone marrow precursor cells that have undergone different differentiation (as histiocytes) under the influence of various environmental (tissue location) and growth factors such as GM-CSF, TNF and IL-4.