To the diagnosis of cerazine reticuloendotheliosis (Gaucher disease)

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Abstract

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.

About the authors

S. L. Luzkova

Hospital therapeutic clinic of the 1st Moscow Order of Lenin Medical Institute named after I.M. Sechenov

Author for correspondence.
Email: info@eco-vector.com
Russian Federation, Moscow

L. N. Komarova

Hospital therapeutic clinic of the 1st Moscow Order of Lenin Medical Institute named after I.M. Sechenov

Email: info@eco-vector.com
Russian Federation, Moscow

F. L. Salimon

Hospital therapeutic clinic of the 1st Moscow Order of Lenin Medical Institute named after I.M. Sechenov

Email: info@eco-vector.com
Russian Federation, Moscow

References

Supplementary files

Supplementary Files
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1. JATS XML
2. Microphoto 1. Spleen of the b-nogo T. - typical Gaucher cells, one- and two-nucleated in large numbers (large increase)

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© 1965 Luzkova S.L., Komarova L.N., Salimon F.L.

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