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Low ADAMTS13 antigen and high ADAMTS13 antibody levels associated with mortality in TTP patients

August 1, 2017

While thrombotic thrombocytopenic purpura (TTP) has a mortality rate of approximately 10% even when treated, predictors of mortality are unknown.  In a prospective cohort registry of 292 TTP patients with 312 episodes of TTP in the United Kingdom, factors associated with mortality were evaluated.  TTP patients with high amounts (top quartile) of a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13) antibody had significantly higher mortality than those in the lowest quartile (16.9% vs. 5.0%, P=0.004).  In addition, those patients with ADAMTS13 antigen level in the lowest quartile (<1.5%) had significantly higher mortality than those in the highest quartile (>11%) (18.0% vs. 3.8%, P=0.005).  Furthermore, patients in the lowest quartile for ADAMTS13 antigen and the highest quartile for ADAMTS13 antibody had an increased mortality rate of 27.3%.  Individuals who presented with elevated troponin levels on hospital admission also had increased mortality.  Careful screening, monitoring and aggressive treatment are needed for those individuals at highest risk of mortality.
Reference:

Alwan, F., Vendramin, C., Vanhoorelbeke, K. et al.  Presenting ADAMTS13 antibody and antigen levels predict prognosis in immune-mediated thrombotic thrombocytopenic purpura.  Blood.  2017; 130: 466-471.

 

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