The basic function and composition of blood is 8% of an adult’s body weight, with females having 4-5 litres and males having 5-6 litres.
The temperature of blood is 38 degrees Celcius and the pH is 7.35-7.45.
The viscosity of blood is 4.5-5.5 times as viscous as water.
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 red blood cell membrane is about 50% protein, 40% lipid bilayer, and 10% carbohydrates that occur only on the external surface.
The red blood cell membrane proteins include Integral Proteins such as Band-3 and Glycophorine, and Extrinsic Proteins like Spectrin, Band-4.1, and Band-4.2.
A genetic defect in NADPH oxidase causes chronic granulomatous disease, characterized by severe, persistent, infections and formation of granulomas.
Neutrophils and monocytes play an important role with both oxygen-independent and oxygen-dependent mechanisms for killing bacteria.
High lysosomal enzyme activities such as myeloperoxidase and NADPH oxidase are present in neutrophils.
The oxygen-independent mechanism involves using pH changes in phagolysosomes and lysosomal enzymes to destroy pathogens.
The rapid consumption of molecular oxygen that accompanies formation of superoxide is referred to as the respiratory burst.
The glycolysis and pentose phosphate pathway is very active in neutrophils.
The oxygen-dependent system includes the enzymes, NADPH oxidase and myeloperoxidase (MPO) that work together in killing bacteria.
Moderate oxidative phosphorylation occurs in neutrophils.
Spherical erythrocytes that are old and cannot pass through the reticular sinusoid structure in the spleen are destroyed and disposed there.
CD11/CD18 integrins in the cell membrane exist in neutrophils.
After internalization of the microorganism has occurred, NADPH oxidase, located in the leukocyte cell membrane, is activated and reduces molecular oxygen from the surrounding tissue into superoxide (O2•), a free radical.
Specific enzymes and proteins are present in neutrophils.
Myeloperoxidase catalyzes the formation of bactericidal hypochlorous acid from peroxide and chloride ion.
NADPH oxidase uses molecular oxygen and NADPH electrons to produce superoxide radicals, which, in turn, can be converted to peroxide by superoxide dismutase.
Erythrocytes have no nucleus and mitochondria, and energy in the form of ATP is supplied directly from glucose metabolism.
Band-3 protein, or anion channel, is mainly anion transporter protein of erythrocyte.
Hereditary Elliptocytosis (HE) is a highly variable red cell membrane disorder with many clinical subtypes, it is autosomal dominant inherited.
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.
Glycophorin is a blood group antigen.
The sodium ion inflow to erythrocytes is accelerated by abnormal proteins, normal disc-shaped cells become macro-micro spherocytic.
Band 1 and Band 2 are Spectrin.
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.
The most used test for diagnosis of Hereditary Spherocytosis is the Red Cell fragility test, also called erytrocytic osmotic resistance test, which is a simple and easy method.
Band-7 protein, Stomatin, is a component of the cytoskeleton.
Band 4.1 and Band 4.2 stabilize the binding between ankyrin and anion channel (Band-3).
Spectrin molecules form a mesh-like pattern in the red cell membrane.
Red cell Membrane Proteins are separated by SDS Polyacrylamide Gel Electrophoresis.
Band-5 protein, Actin, is a component of the cytoskeleton.
Hereditary Spherocytosis (HS) is characterized by the presence of spherocytes, which are the most common cause of inherited chronic hemolysis in Northern Europe.
Hereditary Stomatocytosis is a rare hemolytic disorder, the defect is identified as a deficiency in the membrane protein, stomatin (Band 7), leading to increased water in the red cell.
The red blood cell (RBC) is highly dependent upon glucose as its energy source, glucose is transported through RBC membrane by facilitated diffusion through glucose transporters (GLUT-1).
The Embden-Meyerhof pathway provides 90% of the cellular ATP because red cell metabolism is essentially anaerobic.
In normal conditions, erythrocytes behave like osmometers, according to the increasing and decreasing of osmotic pressure in medium, they swell and shrink.