ADAMTS13 in the New Era of TTP.

Another study showed that all-cause mortality was associated with suboptimal ADAMTS13 activity levels but did not reach statistical significance, probably due to the small sample size of the study [ 110 ].

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Evaluation of ADAMTS13 qualities, such as activity, conformation status, antibodies, and antigen, is highly significant in the TTP diagnosis, as open conformation of ADAMTS13 is a subtle marker of TTP confirmation, in cases where ADAMTS13 activity is borderline low (i.e., 10–20%), or when levels of anti-ADAMTS13 inhibitors remain insignificant [ 11 , 52 ] ( Figure 1 ).

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