The treatment of TTP depends on the possible causes. Among children who suffer from TTP after bloody diarrhea, the condition often resolves itself. Similarly, if the TTP was provoked by certain drugs, such as anti-cancer drugs, the first line of treatment is to discontinue the medication.

Among adults with idiopathic TTP, or TTP that cannot be linked to any single cause, the treatment consists of plasma exchange, which involves infusion of fresh plasma coupled with a procedure that removes platelets (plasmapheresis). Steroid drugs are often included in the treatment regimen. This treatment is extremely successful. Prior to the availability of plasma treatment, up to 90 percent of adult patients with TTP died. Today, this figure is reversed: 90 percent of adult TTP patients can be successfully treated with plasma exchange.

During plasma exchange, the patient’s blood is withdrawn in a process similar to a blood donation. The plasma (the fluid part of blood without the blood cells) is then passed through a cell separator to remove the large VWf structures and autoantibodies. The remaining portion of the concentrated plasma is saved, reconstituted to normal volume with a plasma substitute and returned to the patient as a blood transfusion. According to studies, side effects occur with this treatment in as many as 60 percent of patients. Of the major side effects, most are due to the use of a central venous catheter during the blood withdrawal. They include infections, hemorrhage and venous blood clots.

If no side effects are present, this treatment is repeated daily until blood tests show improvement, usually for 7 to 16 days. It is estimated, however, that between 10 and 20 percent of patients do not respond to this initial treatment. These patients are frequently recommended for more intensive plasma exchange therapy, sometimes undergoing two exchanges a day. If that doesn’t work, physicians may add certain medications to the plasma, which have been shown to aid therapy. Once a normal platelet count has been reached, treatment generally tapers off until a lasting remission is achieved.

As a final resort, removal of the spleen (splenectomy) may be recommended. Although there are some conflicting studies, removal of the spleen has been shown to improve the condition among some patients. With modern plasma exchange techniques, the improved outcomes for this disease have increased, so that 80 to 90 percent of patients now go on to complete recovery. Some fatalities still occur, however, and patients who have had the disorder should be monitored with blood tests and physical examinations for several years. Unfortunately, it is not uncommon for TTP patients to have sudden relapses that require another round of plasma exchange. In fact, some patients may need to deal with these recurrences throughout life.

Since the exact cause of TTP is unknown, there is no known way to prevent the disease. However, there are drugs and toxins (poisons) that are suspected in causing some cases of TTP. They include:

At the present time, research is underway to study and define the connection between TTP and these substances.