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

• Published in: Blood 2019-09, Vol.134 (13), p.1037-1045
• Main Authors: Upreti, Harshvardhan, Kasmani, Jamil, Dane, Kathryn, Braunstein, Evan M., Streiff, Michael B., Shanbhag, Satish, Moliterno, Alison R., Sperati, C. John, Gottesman, Rebecca F., Brodsky, Robert A., Kickler, Thomas S., Chaturvedi, Shruti
• Format: Article
• Language: English
• Subjects: ADAMTS13 Protein - metabolism; Adult; Age Factors; Clinical Trials and Observations; Female; Humans; Incidence; Male; Middle Aged; Prospective Studies; Purpura, Thrombotic Thrombocytopenic - complications; Purpura, Thrombotic Thrombocytopenic - metabolism; Retrospective Studies; Sex Factors; Stroke - etiology; Stroke - metabolism; Treatment Outcome
• ISSN: 0006-4971; 1528-0020
• DOI: 10.1182/blood.2019001056

With timely and effective treatment, most patients with thrombotic thrombocytopenic purpura (TTP) survive the acute TTP 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 of stroke during remission. Of 170 consecutive patients treated for TTP at The Johns Hopkins Hospital from 1995 through 2018, 14 (8.2%) died during the index episode and 19 were observed for less than 1 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 fivefold higher than the expected prevalence of 2.6% from an age- and sex-matched reference population (P = .002). ADAMTS13 activity during remission was measured in 52 patients and was >70% in 44.2%, 40% to 70% in 23.1%, 10% to 39% in 25%, and 70%) and in 27.6% (8 of 29) of patients with low ADAMTS13 activity (≤70%; P = .007). In conclusion, stroke is common after recovery from TTP and is associated with reduced ADAMTS13 activity during remission. •Stroke is more common in TTP survivors than in an age- and sex-matched control population.•Lower ADAMTS13 activity after recovery from TTP is associated with stroke. [Display omitted]