Gaucher's disease, light micrograph

Gaucher's disease, light micrograph

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Caption: Gaucher's disease. Light micrograph of a section through the spleen of a patient with Gaucher's disease. This is an inherited metabolic disorder in which a fatty substance called glucocerebroside accumulates in the spleen, lungs, liver and bone marrow, and sometimes the brain. This is due to an absence of the enzyme glucocerebrosidase (GC). The disease appears in childhood, and some forms are fatal in the first few years. Other forms cause enlargement of the affected organs, weak and painful bones and anaemia. Some forms also cause severe nervous system damage. There is no cure, but enzyme replacement therapy using GC can relieve the symptoms.

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