Erdheim–Chester
disease
It
is also known as polyostotic sclerosing histiocytosisor Erdheim–Chester
syndrome. It is a infrequentillnessconsidered by the irregularincrease of a precise
type of white blood cells called histiocytes, or tissue macrophages
(technically, this illness is called a non-Langerhans-cell histiocytosis). Typically,
onset is in middle age.
Symptoms
Erdheim-Chester
can include many dissimilarschemes in the body and most often touches the long
bones. In order of incidence, it also happens in the kidney, retroperitoneum,
brain, skin,lung, and tissue behind hand the pituitary gland,eyes, and heart
Diagnosis
Histologyand
Radiologic osteosclerosisare the main analytic features.
Treatment
High-dose
Corticosteroid therapy, Surgical debulking, Cyclosporin, Interferon,Chemotherapy.
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