RT Journal Article
SR Electronic
T1 Molecular Action of ADAMTS-13 and Transfusion Therapies of Thrombotic Thrombocytopenic Purpura
JF American Society for Clinical Laboratory Science
JO Clin Lab Sci
FD American Society of Chemistry and Laboratory Science
DO 10.29074/ascls.2020002287
A1 Ketelsen, Billie
YR 2020
UL http://hwmaint.clsjournal.ascls.org/content/early/2023/04/10/ascls.2020002287.abstract
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.