PT  - JOURNAL ARTICLE
AU  - Billie Ketelsen
TI  - Molecular Action of ADAMTS-13 and Transfusion Therapies of Thrombotic Thrombocytopenic Purpura
AID  - 10.29074/ascls.2020002287
DP  - 2020 Aug 01
TA  - American Society for Clinical Laboratory Science
4099  - http://hwmaint.clsjournal.ascls.org/content/early/2023/04/10/ascls.2020002287.short
4100  - http://hwmaint.clsjournal.ascls.org/content/early/2023/04/10/ascls.2020002287.full
AB  - Thrombotic thrombocytopenic purpura (TTP) is a disease that is classified by abnormal functioning of a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS-13), protease. ADAMTS-13 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, whereas the acquired version is more common. The acquired version of TTP is caused by inhibitory and noninhibitory autoantibodies that affect the ADAMTS-13 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 ADAMTS-13 proteases in the circulation while providing the addition of normal functioning ADAMTS-13 to the circulation.