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Your choice of the IHTC Pharmacy Program directly supports your IHTC team and patient services, and activities provided to the hemophilia community. The IHTC Pharmacy Program provides savings to you and your health insurance plan.
Every patient has the right to choose their clotting factor pharmacy provider. The IHTC supports your right of choice and will assist you in making an informed decision.
Qualitative Platelet Disorders
What are platelets?
Platelets are small disc shaped cells which are formed in the bone marrow. Seventy to eighty percent of platelets circulate in the bloodstream while the remainder are stored in the spleen. The average platelet lives about 10 days.
Platelets play a crucial role in helping blood to clot. The primary functions of platelets are adhesion, aggregation, activation and secretion. A defect in any one of these functions will result in an increased tendency to bleed.
Platelet adhesion occurs when there is an injury to a blood vessel. Platelets attach to the lining of the injured blood vessel and begin the first phase of the clotting process
After platelets initially begin to adhere to the wall of an injured blood vessel, additional platelets are called to the site of injury. The platelets begin to aggregate or stick to each other, forming a platelet plug.
Activation and Secretion
Granules inside the platelets function to store and release specific proteins that help platelets aggregate at the site of blood vessel injury.
How are platelet disorders diagnosed?
The diagnosis of a platelet disorder involves several laboratory tests. The first are called screening tests. These include blood tests to look for abnormalities in the size and characteristics of the platelets and a bleeding time to measure of how well the platelets stop bleeding. Other studies may include measurement of platelet aggregation by exposing the platelets to a variety of compounds that normally cause platelets to aggregate or stick together. If these tests are abnormal, further testing of the platelets will be performed using electron microscopy to determine the specific type of structural platelet disorder.
What are the symptoms of a platelet disorder?
The most common symptoms of a platelet disorder are easy bruising, nosebleeds, bleeding of the mouth or gums, heavy menstrual bleeding, postpartum bleeding and bleeding following surgical or invasive procedures.
What is a platelet disorder?
Both quantitative and qualitative platelet disorders can predispose an individual to increased bleeding. A quantitative platelet disorder is a condition in which there are not enough platelets. Platelets are either not being made in sufficient amounts, or are being destroyed more rapidly than normal. A qualitative platelet disorder involves abnormal structure or function of the platelet itself. Although- many conditions can affect the platelet number (quantitative platelet disorder), qualitative platelet disorders are rare. A qualitative platelet disorder may involve missing or defective proteins on the surface of the platelet membrane or a deficiency or abnormality in the platelet granules or their contents (storage pool disorder) which affect platelet function. Many qualitative platelet and storage pool disorders have been described. They vary in how they are inherited, their underlying defect and clinical bleeding complications.