The composition of blood includes White Blood Cells (WBCs), Leucocytes, Granulocytes, Agranulocytes, Neutrophils, Lymphocytes, Eosinophils, Monocytes, and Basophils.
Red Blood Cells (also called erythrocytes) are formed elements of blood, with a shape of a biconcave disc, a volume of 80-100 fL, and an area of about 160 μm2.
The major functions of Red Blood Cells (RBCs) are delivering oxygen to the tissues and disposal of carbon dioxide and protons formed by the tissue metabolism.
The Red Blood Cell Maturation involves six stages of maturation in the red cell series, with each normoblast undergoing four more cycles of cell division.
The Red Blood Cells are under the control of erythropoietin (EPO), a hormone produced by the kidneys, that promotes the formation of red blood cells by the bone marrow.
There are four metabolic pathways that are essential for red cell function: Hexose monophosphate shunt pathway, Embden-Meyerhof glycolytic pathway, Luebering-Rapoport shunt, and methemoglobin reductase pathway.
In Hereditary Stomatocytosis, the intracellular sodium content increases, leading to increased water, causing the red cell to swell and appear as if the red cells have slits or bars in the center, as if the cell is “smiling.”
The membrane defect in Hereditary Elliptocytosis is a disordered or deficient spectrin and proteins commonly associated with the alpha and beta spectrin regions.
The defect in Hereditary Spherocytosis is a deficiency of the key membrane protein, spectrin, and to a lesser degree, a deficiency of membrane protein ankyrin and the minor membrane proteins Band 3 and Band 4.2.
Erythropoietin (EPO), also known as hemopoietin, is secreted by the kidney in response to cellular hypoxia; it stimulates red blood cell production (erythropoiesis) in the bone marrow.
Hereditary Spherocytosis (HS) is characterized by the presence of spherocytes, which are the most common cause of inherited chronic hemolysis in Northern Europe.
In Hereditary Spherocytosis, the sodium ion inflow to erythrocytes is accelerated by abnormal proteins, causing normal disc-shaped cells to become macro-micro spherocytic.