Thrombotic thrombocytopenic purpura is a disease causing clotting in small blood vessels, which can lead to overuse of platelets and spontaneous bleeding or bruising. There are two types, primary and secondary, with blood replacement therapy as a common treatment. The primary form has a long-term survival rate close to 90%, while the secondary form is considered much more dangerous.
Thrombotic thrombocytopenic purpura is a disease that causes clotting in small blood vessels. These clots can be very harmful if they block blood flow to vital organs. People with this disorder lack sufficient amounts of a vital enzyme that helps inhibit an important blood clotting protein. Having so many blood clots can lead to overuse of platelets, which are responsible for clotting, and this can lead to spontaneous bleeding or bruising.
Most symptoms of thrombotic thrombocytopenic purpura result from blood flow restriction, but others can be the result of a lack of platelets in the blood. Some of the more serious symptoms involve the brain. Patients may feel confused and can sometimes speak differently or have hallucinations. They may also have a fast heartbeat, fever and weakness, and may pass out. In addition to the aforementioned bleeding and bruising, platelet deficiency can cause small purple spots that look a bit like a rash.
There are two different types of thrombotic thrombocytopenic purpura. The first type is something people are usually born with and is called primary thrombotic thrombocytopenic purpura. Sometimes people acquire the disorder due to some other illness or trauma. When this happens, it is called secondary thrombotic thrombocytopenic purpura.
Scientists believe that the primary form of thrombotic thrombocytopenic purpura is probably genetically transmitted. They are not sure of the exact mechanism involved in the development of the secondary form. Some of the conditions that can lead to secondary thrombotic thrombocytopenic purpura are pregnancy, bone marrow transplants, human immunodeficiency virus (HIV), and cancer. Some people also develop the disorder from lupus, and there is evidence of a connection with hormone replacement therapy.
Doctors often use blood replacement therapy as a treatment for thrombotic thrombocytopenic purpura. This works because the donors will have the right types of enzymes needed to correct the imbalance in the sufferer’s system. In many cases, this disease occurs episodically, so sufferers may need to repeat this treatment every time an episode occurs.
Both forms of thrombotic thrombocytopenic purpura are very dangerous if left untreated, and in many cases, people don’t realize they have them until they are too sick to be helped. When people receive proper treatment, the primary form has a long-term survival rate close to 90%. The secondary form is considered much more dangerous and patients often die even with appropriate treatment. Doctors aren’t sure about the reasons for this difference.
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