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2012, Número 4

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Rev Cubana Hematol Inmunol Hemoter 2012; 28 (4)


Esferocitosis hereditaria: de la biogénesis a la patogénesis

Garrote-Santana H, Gómez-Pacheco M, Jaime-Fagundo JC, Pavón-Morán V, Martínez-Antuña G

Idioma: Español
Referencias bibliográficas: 50
Paginas: 310-326
Archivo PDF: 289.81 Kb.


RESUMEN

La esferocitosis hereditaria es la anemia hemolítica congénita más frecuente en la población caucásica. Tiene una amplia variabilidad clínica y desde el punto de vista hematológico se caracteriza por anemia y presencia de esferocitos en la lámina periférica. Su base fisiopatológica está determinada por el defecto de algunas de las proteínas que conforman la membrana eritrocitaria, por el efecto del bazo sobre los hematíes anómalos y otros factores. A la luz de los conocimientos actuales, la interpretación dinámica del proceso requiere adentrarse en los estadios iniciales de la hematopoyesis, pues desde etapas tan tempranas como la enucleación del eritroblasto en la formación del reticulocito, hasta posibles procesos inflamatorios tardíos, pudieran modular la expresión de la enfermedad. Se hace una revisión de las características estructurales y funcionales de la membrana eritrocitaria, así como algunos aspectos generales de las propiedades del hematíe para facilitar la comprensión de los eventos que tienen lugar a partir del compromiso molecular de las proteínas que conforman la membrana.


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