Gaucher's Disease

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Abstract
GAUCHER'S disease is an uncommon hereditary metabolic disorder characterized by the accumulation of cerebrosides in the reticuloendothelial system. Some 300 cases have been reported in the literature since the condition was first described by Gaucher in 1882.In the "adult" form, the clinical picture is that of enlargement of the spleen and liver accompanied later by pigmentation of the skin, mainly of the exposed parts, and wedge-shaped, brown thickening of the sclera. A mild anemia, leukopenia and thrombocytopenia with a bleeding diathesis may develop. The infiltration of Gaucher cells may cause pulmonary1 2 3 4 5 6 7 8 and bone involvement,9 , 10 giving characteristic x-ray findings. Gaucher's . . .
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