Gaucher’s disease

Gaucher’s disease

• This lipid storage disease is characterised by storage of glucocerebroside in the reticuloendothelial system and in the spleen. Enormous splenic enlargement may be associated with yellowish brown discoloration of the skin on the hands - and face, anaemia and conjunctival thickening (pinguecula). - Slavonic and Jewish races are more prone to the disease, and the detection of Gaucher cells in the bone marrow confirms the diagnosis. Splenectomy is indicated only for severe symptoms related to the splenomegaly . -