Molecular Action of ADAMTS13 and Transfusion Therapies of Thrombotic Thrombocytopenia Pupura

Abstract

Thrombotic Thrombocytopenia Pupura (TTP) is a disease that is classified by abnormal functioning of the ADAMTS13 protease. ADAMTS13 protease impairment can be caused by genetic mutations at the gene level or through autoantibodies that are formed within the circulation. Congenital mutations account for about 5-10% of the TTP population while the acquired version is more common. The acquired version of TTP is due to inhibitory and non-inhibitory autoantibodies that affect the ADAMTS13 protease. Both congenital and acquired TTP are treated through transfusion therapy with therapeutic plasma exchange (TPE). TPE is used to remove the autoantibodies and any mutated ADAMTS13 proteases in the circulation while providing the addition of normal functioning ADAMTS13 to the circulation.