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 or weeks, but it can go on for months. Relapses (flareups) can occur in up to 60 percent of people who have the acquired type of TTP. Flareups also occur in most people who have 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 who have inherited TTP, and plasma exchange for people who have 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 inserted into a vein (blood vessel). This is done to replace the missing or altered ADAMTS13 enzyme.

For acquired TTP, plasma exchange (also called plasmapheresis) is done. This is a lifesaving procedure. It removes antibodies (proteins) 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 a vein 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 inserted into 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 take days or weeks, depending on your condition. You'll 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 if plasma infusions or plasma exchange doesn't work well, or if 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) sometimes is needed. This is because cells in the spleen make the antibodies that block ADAMTS13 enzyme activity.