Thrombotic thrombocytopenic purpura (TTP) is a blood disorder that causes blood clots to form in blood vessels around the body. TTP can lead to low platelets, low red blood cell count , abnormal kidney function, and problems with the nervous system. TTP can affect any organ system, but involvement of the peripheral blood, the central nervous system, and the kidneys causes the clinical manifestations. Some cases may be caused by a lack of the ADAMTS13 gene at birth. This gene gives the instructions for making the enzyme involved in blood clotting. It affects 4 to 7 per million people each year in the United States. The two major forms of thrombotic thrombocytopenic purpura are an acquired (noninherited) form and a familial form. The acquired form usually appears in late childhood or adulthood.



Plasma exchange (plasmapheresis) is used to remove unwanted substances from the blood and to replace the missing enzyme that normally breaks down proteins (protease). Plasmapheresis may need to be continued for 1-8 weeks before patients with idiopathic TTP cease to consume platelets and begin to normalize their hemoglobin. Corticosteroids (steroids) are used in combination with plasma exchange. Their immunosuppressive effects are thought to be important. Steroids may be given either intravenously or orally. Many TTP patients need additional immunosuppressive therapy, with glucocorticoid steroids (e.g. prednisolone or prednisone), vincristine, cyclophosphamide, splenectomy or a combination of the above. Rituximab, a monoclonal antibody targeting B cells.

It has been successfully used to treat patients with refractory disease. Lactate dehydrogenase levels are generally used to monitor disease activity. Aspirin (eg Micropyrin) is an anti-platelet agent and may be commenced. Folic acid is a vitamin required for healthy formation of red cells. Folic acid is a vitamin required for healthy formation of red cells. The body cannot store large amounts of this vitamin and, in circumstances of excessive red cell production like TTP, folic acid deficiency can develop relatively quickly. Rituximab, a monoclonal antibody targeting B cells, has been successfully used to treat patients with refractory disease.

Vincristine (eg Oncovin) is a chemotherapy drug that may be helpful in the treatment of TTP. Red cell transfusion is also recommended.

Thrombotic Thrombocytopenic Purpura Treatment Tips

1. Steroids may be given either intravenously or orally.

2. Folic acid is a vitamin required for healthy formation of red cells.

3. Lactate dehydrogenase levels are generally used to monitor disease activity.

4. Corticosteroids (steroids) are used in combination with plasma exchange.

5. Vincristine (eg Oncovin) is a chemotherapy drug that may be helpful in the treatment of TTP.

6. Splenectomy (surgical removal of the spleen) has been proposed as treatment for refractory disease.