To the diagnosis of cerazine reticuloendotheliosis (Gaucher disease)
1965
In 1882, Gaucher first described a disease called isolated splenic epithelioma. Later it was proved that this is not an epithelioma or a disease of the spleen, but a violation of lipoid metabolism. As a result of such a violation, the cells of the reticulo-endothelium (spleen, bone marrow, liver, to a lesser extent - lymph nodes) absorb the product of pathological lipoid metabolism - cerazine and turn into Gaucher cells. The second name of the disease is cerazine reticuloendotheliosis. R.D.Stern considers it to be accumulation reticulosis. The etiology of the disease is unknown. The disease is quite rare (50 patients are described in the domestic literature), often has a familial character (34% according to Peak) and is characterized by a long "ultra-chronic" course (more than 20 years). More often women are ill (75% according to Peak). The disease begins mainly in early childhood.
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