Which factors increase the mortality risk of thrombotic thrombocytopenic purpura (TTP)?

Updated: May 25, 2021
  • Author: Theodore Wun, MD, FACP; Chief Editor: Srikanth Nagalla, MD, MS, FACP  more...
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A study by Staley et al of 73 patients with immune-mediated TTP found that the following findings on admission were associated with higher mortality [32] :

  • Prolonged activated partial thromboplastin time
  • High fibrinogen level
  • Elevated serum lactate dehydrogenase level
  • Elevated complement activation marker Bb
  • Elevated complement activation marker sC5b-9

Other findings predictive of higher mortality included the following [32] :

  • Failure to normalize platelet counts within 7 days
  • Failure to markedly reduce serum lactate dehydrogenase by day 5
  • Low total serum protein or albumin
  • High troponin levels prior to total plasma exchange

Shumak et al reported that more than one third of patients who survive an acute episode of TTP will have at least one relapse in the following 10 years. [33] French researchers found that patients who have had acute TTP are at increased risk for development of an autoimmune disorder—most often, systemic lupus erythematosus (SLE) or Sjögren syndrome—for as long as 12 years afterward. Risk was highest in patients who had anti–double-stranded (ds)DNA antibodies (hazard ratio [HR] 4.98) or anti-SSA antibodies (HR 9.98) at the time of TTP diagnosis. These researchers recommend prolonged follow-up to detect any autoimmune disorder early in its course. [34]

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