Hereditary Spherocytosis: Overview; Complications, Managements, Treatments —— Hereditary Spherocytosis

----- 遗传性球细胞增多症:综述;并发症、治疗、治疗:遗传性球细胞增多症

ISBN: 9786138924739 出版年:2020 页码:64 Eman Refaat Youness Scholars' Press

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Hereditary spherocytosis is an abnormality of red blood cells, or erythrocytes. The disorder is caused by mutations in genes relating to membrane proteins that allow for the erythrocytes to change shape. The abnormal erythrocytes are sphere-shaped (spherocytosis) rather than the normal biconcave disk shaped. Dysfunctional membrane proteins are observed as well and interfere with the cell's ability to be flexible to travel from the arteries to the smaller capillaries. This difference in shape also makes the red blood cells more prone to rupture. Cells with these dysfunctional proteins are degraded in the spleen. This shortage of erythrocytes results in hemolytic anemia.

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