Who gets TTP?

It usually affects people between 20 to 50 years of age but people of any age may be affected. TTP is occasionally associated with pregnancy and collagen-vascular diseases (a group of diseases affecting connective tissue).

Which disease is associated with TTP?

Causes. A lack of activity in the ADAMTS13 enzyme (a type of protein in the blood) causes thrombotic thrombocytopenic purpura (TTP). The ADAMTS13 gene controls the enzyme, which is involved in blood clotting.

What causes acquired TTP?

Acquired TTP is caused when a person’s body mistakingly makes antibodies that block the activity of the ADAMTS13 enzyme . THe ADAMTS13 enzyme normally helps control the activity of certain blood clotting factors. Treatment includes plasma exchange and in some cases may also include corticosteroid therapy or rituximab.

Is TTP inherited or acquired?

Most cases of TTP are acquired, caused by autoantibody-mediated inhibition or clearance of ADAMTS13 activity. Hereditary TTP, caused by pathogenic variants in the ADAMTS13 gene, is much less common but no less life-threatening.

Can a person with TTP have blood clotting problems?

In patients with TTP the ADAMTS 13 enzyme cannot work properly, putting the patient at high risk of blood clotting problems. TTP only affects around one person per one million and is more common in women than men. It can affect people at any age.

What does TTP stand for in medical terms?

TTP describes an illness for which the full name is too long to remember: T is for Thrombotic, a term describing blood clots, which are called thrombi. In TTP, thrombi caused by clumps of platelets block small blood vessels. That can cause damage to organs such as the kidneys, heart, and brain.

Which is the most effective treatment for TTP?

Beginning in the 1970s, plasma exchange (PEX) was first recognized as an effective treatment for TTP. In 1991, PEX was documented to be effective, with approximately 80% of patients survining. Since 1991, PEX has become the standard treatment for TTP.

How are platelets used up in TTP patients?

In TTP, they are used up in the abnormal clots that occur throughout the body. P is for Purpura, a term describing the bruises and the small purple bleeding spots that are caused by too few platelets. The picture below is a microscopic view of blood cells from a patient with TTP. Notice that there are no platelets.