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Encyclopedia > Glanzmann's thrombasthenia

Glanzmann's thrombasthenia is an extremely rare, autosomal recessive disorder of the blood, in which the platelets lack GPIIb/IIIa. Hence, no fibrinogen bridging can occur, and bleeding time is significantly prolonged. An autosome is a non-sex chromosome. ... In genetics, the term recessive gene refers to an allele that causes a phenotype (visible or detectable characteristic) that is only seen in a homozygous genotype (an organism that has two copies of the same allele). ... Blood diseases affect the production of blood and its components, such as blood cells, hemoglobin, blood proteins, the mechanism of coagulation, etc. ... Red blood cells (erythrocytes) are present in the blood and help carry oxygen to the rest of the cells in the body Blood is a circulating tissue composed of fluid plasma and cells (red blood cells, white blood cells, platelets). ... A 250 ml bag of newly collected platelets. ... Fibrin is a protein involved in the clotting of blood. ...


The GpIIb/IIIa is a adhesion receptor and is expressed in thrombocytes. This receptor is activated when the thrombocyte is stimulated by ADP, epinephrine, collagen and thrombin. The GpIIb/IIIa integrin is essential to the blood coagulation since it has the ability to bind fibrinogen, the von Willebrand factor, fibronectin and vitronectin. This enables the platelet to be activated by contact with the collagen-von Willebrand-complex that is exposed when the endothelial blood vessel lining is damaged. ... ADP may stand for: adenosine diphosphate Aeroports de Paris [1] AOLserver Dynamic Pages, web server extensions designed to return dynamically created documents based on the TCL computer programming language using AOLserver. ... Epinephrine (INN) or adrenaline (BAN) is a hormone and a neurotransmitter. ... Collagen is the main protein of connective tissue. ... Thrombin (activated Factor II) is a coagulation protein that has many effects in the coagulation cascade. ... An integrin, or integrin receptor, is an integral membrane protein in the plasma membrane of cells. ... Coagulation is the thickening or congealing of any liquid into solid clots. ... Fibrin is a protein involved in the clotting of blood. ... Von Willebrand factor (vWF, also called Factor VIII-related antigen) is a blood protein of the coagulation system. ... Fibronectin is high molecular weight glycoprotein containing about 5% carbohydrate that bind to receptor proteins spanning the cell membrane called integrins and to the extracellular matrix. ...


Patients suffering from Glanzmann's thrombasthenia thus have platelets less able to adhere to the underlying tissue of damaged blood vessels.


Clinical Features

Characteristically, there is increased mucosal bleeding:

The bleeding tendency is variable but may be severe. Nosebleed as a result of fracture through a rugby impact. ... Menorrhagia is an abnormally heavy and prolonged menstrual periods. ...


Patients present with moderate bleeding and normal platelet morphology. Aggregation of platelets occurs in response to ristocetin, but not to other agonists such as ADP, thrombin, collagen or adrenalin. Bleeding is the loss of blood from the body. ... Look up Aggregation in Wiktionary, the free dictionary The term aggregation may refer to— in economics, any attempt to bypass information asymmetry by systematically amassing information about prices and other features of goods on the market. ... ADP may stand for: adenosine diphosphate Aeroports de Paris [1] AOLserver Dynamic Pages, web server extensions designed to return dynamically created documents based on the TCL computer programming language using AOLserver. ... Thrombin (activated Factor II) is a coagulation protein that has many effects in the coagulation cascade. ... Collagen is the main protein of connective tissue. ... Epinephrine (INN) or adrenaline (BAN) is a hormone and a neurotransmitter. ...


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