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Thrombogenic von Willebrand factor released from stimulated endothelial cells is cleaved and inactivated by ADAMTS13, a plasma protease. Patients with immune thrombotic thrombocytopenic purpura (TTP) have antibodies to ADAMTS13 and experience widespread ischemic organ damage.
To identify factors associated with a poor prognosis in TTP, investigators analyzed U.K. TTP registry data collected from 292 patients during a 7-year period.
The following results were reported:
The overall mortality rate was 10.3%, and the median time to death from first presentation was 4 days.
Median ADAMTS13 antibody levels were higher in those who died versus those who survived (66% vs. 39%; P=0.004); median ADAMTS13 antigen levels were lower in those who died versus…