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Thrombotic Thrombocytopenic Purpura

Treatment and Management

Plasmapheresis is the standard of care: IV plasma exchange, also called plasmapheresis, is the most effective therapy, exchange fresh frozen plasma one to two times the estimated plasma volume or 40-80 ml/kg, up to 4.0 L. Plasma infusion of fresh frozen plasma is used as a temporizing measure until the patient can be transferred to a facility where plasma exchange is available. Plasma exchange is used to remove unwanted substances from the blood (antigen antibody complexes). Blood is withdrawn from the patient and the plasma portion of the blood is passed through a cell separator. The remaining portion of the blood is saved, reconstituted with a plasma substitute, and returned to the patient as a blood transfusion. This treatment is repeated daily until blood tests show improvement.

The standard replacement fluid is fresh frozen plasma; however, cryosupernatant has been reported successfully. Cryosupernatant is the residual plasma fraction after separation of cryoprecipitate. It is an excellent treatment and is sometimes preferable for therapeutic use.

People who do not respond to this treatment, or who have frequent recurrences may require the removal of the spleen. Some patients benefit from splenectomy, presumably because the spleen is a major site of autoantibody production. Another possibility is that the spleen is a major site of microvascular occlusive lesions in severe TTP.

Steroids and antiplatelet agents are used. Steroids are often used prior to plasma exchange. In the United States, almost all patients receive prednisone, predinisolone, or methylprednisolone--in association with plasma exchange.

A few adults with refractory single episodes of TTP, or with frequent recurrences to immunosuppressive therapy, are typically treated with azathioprine. Perhaps half of the patients in whom plasma exchange is ineffective will respond to either splenectomy or immunosuppression. Platelet transfusion is contraindicated since it is associated with rapid deterioration of the patient's condition.