One of the materials that helps platelets to stick together to form a clot is a protein called von Willebrand Factor vWF. You can think of vWF as the sticky substance that platelets attach to in order to form a clot. This is because large molecules of vWF attract platelets at a very high rate, forming blood clots even though there is no bleeding and reducing the number of platelets circulating in the blood. Without functioning ADAMTS13, the blood clotting process goes into overdrive, with small clots forming spontaneously throughout the body.
A low number of platelets in the blood a condition called thrombocytopenia can cause bleeding problems, such as bleeding inside the body or underneath the skin. The formation of blood clots in small blood vessels throughout the body However, it is not known why the body starts to produce these antibodies.
Although the cause is not fully understood, there are certain triggers that may be linked to TTP in some patients. These include 6 :. Each TTP patient is different and they may experience a wide range of symptoms, because the entire body is affected by the disease. Many patients may initially experience general, non-specific symptoms such as fever or flu-like symptoms. All rights reserved.
Welcome About TTP. Thrombotic thrombocytopenic purpura TTP is a rare blood disorder. Key points. Introduction to TTP. These blood tests often include tests demonstrating that red blood cells are being destroyed, tests to see how well the kidneys are working, and often tests to detect the presence of antibodies against the ADAMTS13 enzyme. With thrombotic thrombocytopenic purpura TTP , people are often treated with corticosteroids and plasma exchange plasma transfusions along with plasmapheresis Plateletpheresis platelet donation In addition to normal blood donation and transfusion, special procedures are sometimes used.
In plateletpheresis, a donor gives only platelets rather than whole blood. Whole blood is drawn from In plasmapheresis, blood is taken from the person and put in a machine that separates the blood cells from the liquid part of the blood plasma. The plasma, which contains disease-causing antibodies, is discarded and the blood cells are returned to the person. Rituximab is a drug that suppresses the immune system and is sometimes used in people with TTP who relapse after being treated with corticosteroids and plasmapheresis.
Caplacizumab is a new drug that inhibits the interaction between a protein involved in clotting and platelets. Although caplacizumab may reduce the need for plasma exchange and speed recovery in TTP, it may increase the risk of bleeding. Merck and Co. From developing new therapies that treat and prevent disease to helping people in need, we are committed to improving health and well-being around the world. The Manual was first published in as a service to the community.
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Common Health Topics. Platelet Disorders. Test your knowledge. What is the medical term for the procedure in which a person donates his or her own blood to be used if needed during or after a surgical procedure? More Content. Your doctor may begin by performing a physical exam.
This involves looking for any of the physical symptoms of the disease. Plasma is the liquid portion of blood that contains essential clotting factors. You can receive it in the form of fresh frozen plasma. This means that plasma from a healthy donor replaces your plasma. During this procedure, a healthcare professional will draw your blood, just like when you donate blood. Afterward, a lab technician will separate the plasma from your blood using a special machine called a cell separator.
The donated plasma contains water, proteins, and essential clotting factors. This procedure takes about 2 to 4 hours. In other cases, your spleen might need to be surgically removed.
Removal of the spleen can help increase your platelet count. Between 80 and 90 percent of people survive after receiving proper treatment. For some people, TTP goes away completely after treatment.
For other people, they may have ongoing flare-ups. Purpura refers to purple-colored spots that are most recognizable on the skin. Purpura spots, also called blood spots or skin hemorrhages, are…. A creatinine blood test measures the level of creatinine, a waste product, in the blood.
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