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
SP 32
OP 36
DO 10.29074/ascls.2020002287
VO 33
IS 3
A1 Ketelsen, Billie
YR 2020
UL http://hwmaint.clsjournal.ascls.org/content/33/3/32.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.