How Is Thrombotic Thrombocytopenic Purpura Treated?

Thrombotic thrombocytopenic purpura (TTP) can be fatal or cause lasting damage, such as brain damage or stroke, if it’s not treated promptly.

In most cases, TTP occurs suddenly and lasts for days and weeks, but it can go on for months. Most people recover fully when treated right away. Relapses (flareups) can occur in 30 to 60 percent of people with acquired TTP. Flareups also occur in most people with inherited TTP.

Plasma treatments are the most common way to treat TTP. Other treatments include medicines and surgery. Treatments are done in a hospital.

Plasma Therapy

Plasma is the liquid part of your blood. It carries blood cells, hormones, enzymes, and nutrients to your body.

TTP is treated with plasma therapy. This includes fresh frozen plasma for newborns and children with inherited TTP, and plasma exchange for people with acquired TTP. Plasma therapy is started in the hospital as soon as TTP is diagnosed or suspected.

For inherited TTP, fresh frozen plasma is given through an intravenous (IV) line in a vein (blood vessel). This is done to replace the missing or changed ADAMTS13 enzyme.

For acquired TTP, plasma exchange (also called plasmapheresis) is done. This is a lifesaving procedure. It removes antibodies from the blood that damage your ADAMTS13 enzyme. It also replaces the ADAMTS13 enzyme. If plasma exchange isn’t available, you may be given fresh frozen plasma until it is available.

During plasma exchange, an IV needle or tube is placed in your arm to remove blood. The blood then goes through a cell separator, which removes plasma from the blood. The nonplasma part of the blood is saved, and donated plasma is added to it.

The blood is then put back into you through an IV line in one of your blood vessels. The time the procedure takes varies, but it often takes about 2 hours.

Treatments of fresh frozen plasma or plasma exchange usually continue until your blood tests and signs and symptoms improve. This can be days or weeks depending on your condition. You will stay in the hospital during this time.

Some people who recover from TTP have flareups. This can happen in the hospital or after you go home. If you have a flareup, plasma infusion or plasma exchange will be restarted.

Other Treatments

Other treatments are used when plasma infusions or plasma exchange doesn’t work well or when flareups occur often.

Medicines are used to slow or stop antibodies from forming. In acquired TTP, antibodies block the activity of the ADAMTS13 enzyme. Medicines used to treat TTP include glucocorticoids, vincristine, rituximab, and cyclosporine A.

Surgery to remove the spleen (an organ in the abdomen) is sometimes needed. This is because cells in the spleen make antibodies that block the activity of the ADAMTS13 enzyme.