Category: B

Bernard–Soulier syndrome (BSS), also called hemorrhagiparous thrombocytic dystrophy, is a rare autosomal recessive coagulopathy (bleeding disorder) that causes a deficiency of glycoprotein Ib (GpIb), the receptor for von Willebrand factor, an important glycoprotein involved in hemostasis.

The incidence of BSS is estimated to be less than 1 case per million persons, based on cases reported from Europe, North America, and Japan.

BSS is a giant platelet disorder, meaning that it is characterized by abnormally large platelets.

Bleeding, technically known as hemorrhaging or haemorrhaging (see American and British spelling differences), is blood escaping from the circulatory system. Bleeding can occur internally, where blood leaks from blood vessels inside the body, or externally, either through a natural opening such as the mouth, nose, ear, urethra, vagina or anus, or through a break in the skin. Hypovolemia is a massive decrease in blood volume, and death by excessive loss of blood is referred to as exsanguination. Typically, a healthy person can endure a loss of 10–15% of the total blood volume without seriousmedical difficulties (by comparison, blood donation typically takes 8–10% of the donor’s blood volume). The stopping or controlling of bleeding is called hemostasis and is an important part of both first aid and surgery.

Blood is a bodily fluid in animals that delivers necessary substances such as nutrients and oxygen to the cells and transports metabolic waste products away from those same cells. When it reaches the lungs, gas exchange occurs when carbon dioxide is diffused out of the blood into the pulmonary alveoli and oxygen is diffused into the blood. This oxygenated blood is pumped to the left hand side of the heart in the pulmonary vein and enters the left atrium. From here it passes through the mitral valve, through the ventricle and taken all around the body by the aorta. Blood containsantibodies, nutrients, oxygen and much more to help the body work.

In vertebrates, it is composed of blood cells suspended in blood plasma. Plasma, which constitutes 55% of blood fluid, is mostly water (92% by volume), and contains dissipated proteins, glucose, mineral ions, hormones, carbon dioxide (plasma being the main medium for excretory product transportation), and blood cells themselves. Albumin is the main protein in plasma, and it functions to regulate the colloidal osmotic pressure of blood. The blood cells are mainly red blood cells (also called RBCs or erythrocytes) and white blood cells, including leukocytes and platelets. The most abundant cells in vertebrate blood are red blood cells. These contain hemoglobin, an iron-containing protein, which facilitates oxygen transport by reversibly binding to this respiratory gas and greatly increasing its solubility in blood. In contrast, carbon dioxide is almost entirely transported extracellularly dissolved in plasma as bicarbonate ion.

Vertebrate blood is bright red when its hemoglobin is oxygenated and dark red when it is deoxygenated. Some animals, such as crustaceans and mollusks, use hemocyanin to carry oxygen, instead of hemoglobin. Insects and some mollusks use a fluid called hemolymph instead of blood, the difference being that hemolymph is not contained in a closedcirculatory system. In most insects, this “blood” does not contain oxygen-carrying molecules such as hemoglobin because their bodies are small enough for their tracheal system to suffice for supplying oxygen.

Jawed vertebrates have an adaptive immune system, based largely on white blood cells. White blood cells help to resist infections and parasites. Platelets are important in the clotting of blood. Arthropods, using hemolymph, have hemocytesas part of their immune system.

Blood is circulated around the body through blood vessels by the pumping action of the heart. In animals with lungs,arterial blood carries oxygen from inhaled air to the tissues of the body, and venous blood carries carbon dioxide, a waste product of metabolism produced by cells, from the tissues to the lungs to be exhaled.

Medical terms related to blood often begin with hemo- or hemato- (also spelled haemo- and haemato-) from the Greekword αἷμα (haima) for “blood”. In terms of anatomy and histology, blood is considered a specialized form of connective tissue, given its origin in the bones and the presence of potential molecular fibers in the form of fibrinogen.

A blood cell, also called a hematocyte, is a cell produced by hematopoiesis and normally found in blood. In mammals, these fall into three general categories:

• Red blood cells – Erythrocytes
• White blood cells – Leukocytes
• Platelets – Thrombocytes.

Together, these three kinds of blood cells add up to a total 45% of the blood tissue by volume, with the remaining 55% of the volume composed of plasma, the liquid component of blood. This volume percentage (e.g., 45%) of cells to total volume is called hematocrit, determined by centrifuge or flow cytometry. Hemoglobin (the main component of red blood cells) is an iron-containing protein that facilitates transportation of oxygen from the lungs to tissues and carbon dioxide from tissues to the lungs.

Blood plasma is the pale yellow liquid component of blood that normally holds the blood cells in whole blood in suspension; this makes plasma the extracellular matrix of blood cells. It makes up about 55% of the body’s total blood volume. It is the intravascular fluid part of extracellular fluid (all body fluid outside of cells). It is mostly water (up to 95% by volume), and contains dissolved proteins (6–8%) (i.e.—serum albumins, globulins, and fibrinogen), glucose, clotting factors, electrolytes (Na+, Ca2+, Mg2+, HCO3−, Cl−, etc.), hormones, and carbon dioxide (plasma being the main medium for excretory product transportation). Plasma also serves as the protein reserve of the human body. It plays a vital role in an intravascular osmotic effect that keeps electrolytes in balanced form and protects the body from infection and other blood disorders.

Blood plasma is prepared by spinning a tube of fresh blood containing an anticoagulant in a centrifuge until the blood cells fall to the bottom of the tube. The blood plasma is then poured or drawn off. Blood plasma has a density of approximately 1025 kg/m3, or 1.025 g/ml.

Blood serum is blood plasma without clotting factors; in other words, “pure” blood. Plasmapheresis is a medical therapy that involves blood plasma extraction, treatment, and reintegration.

Blood vessels are the part of the circulatory system that transports blood throughout the human body. There are three major types of blood vessels: the arteries, which carry the blood away from the heart; the capillaries, which enable the actual exchange of water and chemicals between the blood and the tissues; and the veins, which carry blood from the capillaries back toward the heart. The word vascular, meaning relating to the blood vessels, is derived from the Latin vas, meaning vessel. Avascular refers to being without (blood) vessels.

The blood vessels are the part of the circulatory system that transports blood throughout the human body. There are three major types of blood vessels: the arteries, which carry the blood away from the heart; the capillaries, which enable the actual exchange of water and chemicals between the blood and the tissues; and the veins, which carry blood from the capillaries back toward the heart. The word vascular, meaning relating to the blood vessels, is derived from the Latin vas, meaning vessel. Avascular refers to being without (blood) vessels.