Reduced ADAMTS13 activity during TTP remission is associated with stroke in TTP survivors

Harshvardhan Upreti, Jamil Kasmani, Kathryn Dane, Evan M. Braunstein, Michael B. Streiff, Satish Shanbhag, Alison R. Moliterno, C. John Sperati, Rebecca F. Gottesman, Robert A. Brodsky, Thomas S. Kickler and Shruti Chaturvedi

Key Points

  • Stroke is more common in TTP survivors than in a age- and sex-matched reference US population.

  • Lower ADAMTS13 activity during clinical remission after recovery from TTP is associated with stroke.


With timely and effective treatment, most patients with thrombotic thrombocytopenic purpura (TTP) survive their acute episode. In addition to the risk of relapse, TTP survivors have higher all-cause mortality than the general population and increased rates of chronic morbidities including hypertension, depression, and mild cognitive impairment. We conducted this retrospective-prospective cohort study to determine the incidence and prevalence of stroke after recovery from acute TTP and to test the hypothesis that lower ADAMTS13 activity after recovery from TTP is associated with an increased risk for stroke during remission. Of 170 consecutive patients treated for TTP at the Johns Hopkins Hospital between 1995 and 2018, 14 (8.2%) died during the index episode and 19 were followed for less than one month after recovery. Of the remaining 137 patients, 18 (13.1%) developed stroke unrelated to an acute TTP episode over a median observation period of 3.08 years, which is 5-fold higher than the expected prevalence of 2.6% from an age- and sex-matched reference population (P= 0.002). ADAMTS13 activity during remission was measured in 52 patients and was >70% in 44.2%, 40-70% in 23.1%, 10-39% in 25%, and <10% in 7.7%. Stroke after recovery from acute TTP occurred in 0% (0 of 22) patients with normal remission ADAMTS13 activity (>70%) and in 27.6% (8 of 29) patients with low ADAMTS13 activity ({less than or equal to} 70%) (P=0.007). In conclusion, stroke is common after recovery from TTP and is associated with reduced ADAMTS13 activity during remission.

  • Submitted April 9, 2019.
  • Revision received August 19, 2019.
  • Accepted August 12, 2019.