Resumo

GARROTE-SANTANA, Heidys et al. Hereditary spherocytosis: from biogenesis to pathogenesis. Rev Cubana Hematol Inmunol Hemoter [online]. 2012, vol.28, n.4, pp. 310-326. ISSN 0864-0289.

Hereditary spherocytosis is the most common congenital hemolytic anemia among Caucasian population. It has wide clinical variety and from the haematological point of view, it is characterized by the presence of spherocytes anemia in peripheral lamina. Its pathophysiological defect is determined by some of the proteins that make up the red cell membrane due to the effect on erythrocytes of abnormal spleen, and other factors. In view of current knowledge, the dynamic interpretation of this process requires delving into the early stages of hematopoiesis, since the expression of this disease could modulate from early stages of erythroblast enucleation in reticulocyte formation until late potential inflammatory processes. A review was made on the structural and functional characteristics of the erythrocyte membrane, as well as some general aspects of the properties of the red cell to facilitate understanding of events which take place through proteins molecular involvement forming the membrane.

Palavras-chave : hereditary spherocytosis; erythrocyte membrane; membrane proteins.

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