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MediSur
versão On-line ISSN 1727-897X
Resumo
SARDINAS PONCE, Raysy; GUERRA LEAL, Obel Alcides e VERDECIA, Servando Roig. Giant splenomegaly due to Gaucher disease. Medisur [online]. 2021, vol.19, n.6, pp. 1021-1027. Epub 30-Dez-2021. ISSN 1727-897X.
Gaucher disease is the most common disease of the lysosomal storage diseases group comprised within innate errors of metabolism. There is glucocerebrosides accumulation in different organs and as a consequence, the development of symptoms and multisystemic signs that are established in a chronic and progressive way, such as: visceromegaly, bone destruction and peripheral cytopenias. A 43-years-old male patient is presented with a diagnosis of this disease since childhood, who developed giant splenomegaly with hypersplenism, for which he required splenectomy, obtaining good results with surgical treatment.
Palavras-chave : Gaucher´s disease; splenomegaly; splenectomy.