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Platelet storage pool deficiency is a family of clotting disorders characterized by deficient granules in platelets. Individuals with these disorders have too few or abnormally functioning alpha granules , delta granules , or both alpha and delta granules and are therefore unable to form effective clots, which leads to prolonged bleeding.
Light transmission aggregometry is widely accepted as the gold standard diagnostic tool for assessing platelet function, and a result of absent aggregation with any agonist except ristocetin is highly specific for Glanzmann's thrombasthenia. [9] Following is a table comparing its result with other platelet aggregation disorders:
Von Willebrand disease (VWD) is the most common hereditary blood-clotting disorder in humans. An acquired form can sometimes result from other medical conditions. [1] It arises from a deficiency in the quality or quantity of von Willebrand factor (VWF), a multimeric protein that is required for platelet adhesion.
Low levels of platelets in turn may lead to prolonged or excessive bleeding. It is the most common coagulation disorder among intensive care patients and is seen in a fifth of medical patients and a third of surgical patients. [3] A normal human platelet count ranges from 150,000 to 450,000 platelets/microliter (μL) of blood. [4]
Giant platelet disorder; Two giant platelets (stained purple) are visible in this image from a light microscope (40×) from a peripheral blood smear surrounded by red blood cells. One normal platelet can be seen in the upper left side of the image (purple) and is significantly smaller in size than the red blood cells (stained pink). Specialty ...
Von Willebrand factor (VWF) (German: [fɔn ˈvɪləbʁant]) is a blood glycoprotein that promotes primary hemostasis, specifically, platelet adhesion.It is deficient and/or defective in von Willebrand disease and is involved in many other diseases, including thrombotic thrombocytopenic purpura, Heyde's syndrome, and possibly hemolytic–uremic syndrome. [5]
Disorders of platelet function can be congenital or acquired. Most of these disorders are mild and may respond to therapy with desmopressin (dDAVP). Transfusion is not necessarily required. However, with some more severe disorders such as Glanzmann thrombasthenia, transfusions with large amount of platelets may be needed.
Thrombotic thrombocytopenic purpura (TTP) is a blood disorder that results in blood clots forming in small blood vessels throughout the body. [2] This results in a low platelet count, low red blood cells due to their breakdown, and often kidney, heart, and brain dysfunction. [1]
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