Key Points

• Thrombotic thrombocytopenic purpura (TTP) is a rare blood condition. It causes blood clots to form in small blood vessels throughout the body. The blood clots can cause serious problems if they block the blood vessels and limit blood flow to the brain, kidneys, or heart.
• Blood clots form when blood cell fragments called platelets clump together. When this happens, the result is fewer platelets in the blood. This can cause bleeding into the skin, prolonged bleeding from cuts, and internal bleeding.
• TTP also can cause red blood cells to break apart faster than the body can replace them. This leads to hemolytic anemia.
• The two main types of TTP are inherited and acquired (noninherited). Inherited TTP mainly affects newborns and children. Acquired TTP occurs mostly in adults, but sometimes affects children.
• A lack of activity in the ADAMTS13 enzyme (a type of protein in the blood) causes TTP. The ADAMTS13 gene controls the enzyme, which is involved in blood clotting. Not having enough enzyme activity causes platelets to clump together and form blood clots.
• What triggers TTP isn't known, but some diseases or conditions, medical procedures, medicines, and substances may contribute to it.
• Most cases of TTP are the acquired type. The condition occurs more often in women and Blacks than in other groups.
• Signs and symptoms of TTP include purplish bruises on the skin or mucous membranes called purpura, pinpoint red or purple dots on the skin called petechiae, paleness or jaundice (a yellowish color of the skin or whites of the eyes), fatigue (tiredness and weakness), fever, a fast heart rate or shortness of breath, headache, speech changes, confusion, coma, stroke, seizure, a low amount of urine, or protein or blood in the urine.
• Your doctor will diagnose TTP based on your medical history, a physical exam, and test results.
• TTP usually is treated with infusions of fresh frozen plasma or plasma exchange. Other treatments, such as medicines and surgery, are used when plasma treatment doesn't work well or relapses (flareups) occur often.
• Both inherited and acquired TTP occur suddenly with no clear cause. You can't prevent either type.
• You should call your doctor right away or seek emergency help if you have signs or symptoms of a TTP flareup. These are the same as the initial signs and symptoms of TTP.
• You also should report any symptoms of infection. Talk with your doctor about changing medicines that may raise your risk of TTP or bleeding.
• Some people fully recover from TTP. However, flareups can occur in up to 60 percent of people who have the acquired type of TTP. They also occur in most people who have inherited TTP.