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Why is electrophoresis with hemoglobin component of 86.5% HbS key test for sickle cell anemia? How...

Why is electrophoresis with hemoglobin component of 86.5% HbS key test for sickle cell anemia? How is sickle cell anemia inherited? Why would reticulocyte percentage increase during a sickle cell attack?

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In sickle cell anemia, RBC becomes an elongated, rigid and sickle shape. These sickle cells obstruct the small blood vessels due to their shape. This results in vascular occlusions and minor infractions. This leads to the periodic painful crisis and also the permanent damage to the tissues and organs.

In-gel electrophoresis, gel (eg: agarose) is used to forms a limiting matrix, i.e. of different pore size. The molecules are separated based on their size over a period of time. The hemoglobin component of 86.5% HbS is a key test in sickle-cell diagnosis because of the size shape of sickle cells different from the normal cells.

Sickle cell anemia is an autosomal recessive disorder of the hematopoietic system, in which the red blood cells become sickle-shaped and cannot carry oxygen. The symptoms include the decreased hemoglobin, high reticulocyte count and bilirubin levels. The high bilirubin is due to the damage of abnormal RBC. Sickle cell trait is the heterozygous condition. In this less than half of the hemoglobin contains abnormal HbS.

Clinical signs will have occurred only in the condition of severe hypoxic conditions like high altitudes or pneumonia. It induces increased production of reticulocytes due to the reduced oxygen-carrying capacity of the blood.

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