Cardiac troponin-I on diagnosis predicts early death and refractoriness in acquired thrombotic thrombocytopenic purpura. Experience of the French Thrombotic Microangiopathies Reference Center

J Thromb Haemost. 2015 Feb;13(2):293-302. doi: 10.1111/jth.12790. Epub 2014 Dec 18.


Background: Cardiac involvement is a major cause of mortality in patients with thrombotic thrombocytopenic purpura (TTP). However, diagnosis remains underestimated and delayed, owing to subclinical injuries. Cardiac troponin-I measurement (cTnI) on admission could improve the early diagnosis of cardiac involvement and have prognostic value.

Objectives: To assess the predictive value of cTnI in patients with TTP for death or refractoriness.

Patients/methods: The study involved a prospective cohort of adult TTP patients with acquired severe ADAMTS-13 deficiency (< 10%) and included in the registry of the French Reference Center for Thrombotic Microangiopathies. Centralized cTnI measurements were performed on frozen serum on admission.

Results: Between January 2003 and December 2011, 133 patients with TTP (mean age, 48 ± 17 years) had available cTnI measurements on admission. Thirty-two patients (24%) had clinical and/or electrocardiogram features. Nineteen (14.3%) had cardiac symptoms, mainly congestive heart failure and myocardial infarction. Electrocardiogram changes, mainly repolarization disorders, were present in 13 cases. An increased cTnI level (> 0.1 μg L(-1) ) was present in 78 patients (59%), of whom 46 (59%) had no clinical cardiac involvement. The main outcomes were death (25%) and refractoriness (17%). Age (P = 0.02) and cTnI level (P = 0.002) showed the greatest impact on survival. A cTnI level of > 0.25 μg L(-1) was the only independent factor in predicting death (odds ratio [OR] 2.87; 95% confidence interval [CI] 1.13-7.22; P = 0.024) and/or refractoriness (OR 3.03; 95% CI 1.27-7.3; P = 0.01).

Conclusions: A CTnI level of > 0.25 μg L(-1) at presentation in patients with TTP appears to be an independent factor associated with a three-fold increase in the risk of death or refractoriness. Therefore, cTnI level should be considered as a prognostic indicator in patients diagnosed with TTP.

Keywords: ADAMTS13 protein, human; prognosis; thrombotic microangiopathy; thrombotic thrombocytopenic purpura; troponin.

Publication types

  • Evaluation Study
  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • ADAM Proteins / deficiency
  • ADAM Proteins / genetics
  • ADAMTS13 Protein
  • Adult
  • Aged
  • Biomarkers / blood
  • Chi-Square Distribution
  • Electrocardiography
  • Female
  • France
  • Heart Diseases / blood*
  • Heart Diseases / diagnosis
  • Heart Diseases / etiology*
  • Heart Diseases / mortality
  • Humans
  • Logistic Models
  • Male
  • Middle Aged
  • Multivariate Analysis
  • Odds Ratio
  • Predictive Value of Tests
  • Prognosis
  • Prospective Studies
  • Purpura, Thrombotic Thrombocytopenic / blood*
  • Purpura, Thrombotic Thrombocytopenic / complications*
  • Purpura, Thrombotic Thrombocytopenic / diagnosis
  • Purpura, Thrombotic Thrombocytopenic / genetics
  • Purpura, Thrombotic Thrombocytopenic / mortality
  • Registries
  • Risk Factors
  • Time Factors
  • Troponin I / blood*
  • Up-Regulation


  • Biomarkers
  • Troponin I
  • ADAM Proteins
  • ADAMTS13 Protein
  • ADAMTS13 protein, human

Supplementary concepts

  • Thrombotic thrombocytopenic purpura, acquired