Gaucher's Disease

In spite of the comparative rarity of the condition, Gaucher's disease has of late aroused a considerable amount of interest, chiefly because of a rather unique pathologic process in the bone. From the present state of knowledge concerning its pathogenesis and evolution, we may define the condition as a familial, chronic, constitutional, nonhereditary disease of metabolism, which is characterized by the deposition of cerebroside kerasin in certain cells of the reticulo-endothelial system. Clinically, it shows splenohepatomegaly, without ascites, occasional lymphadenopathy, subicteric pigmentation of the exposed parts of the skin, pinguecula-like thickening of the ocular conjunctiva, hemorrhagic diathesis, unique changes in the bones, a hypochromic type of anemia, slight but early leukopenia, frequent thrombocytopenia and spastic irritative contractions and tremors of a central type. The disease is considered rare, although the increasing knowledge of the condition is causing more frequent recognition of it in recent years. A comprehensive tabulation of all