TY - JOUR
T1 - Reduced ADAMTS13 activity during TTP remission is associated with stroke in TTP survivors
AU - Upreti, Harshvardhan
AU - Kasmani, Jamil
AU - Dane, Kathryn
AU - Braunstein, Evan M.
AU - Streiff, Michael B.
AU - Shanbhag, Satish
AU - Moliterno, Alison R.
AU - John Sperati, C.
AU - Gottesman, Rebecca F.
AU - Brodsky, Robert A.
AU - Kickler, Thomas S.
AU - Chaturvedi, Shruti
N1 - Publisher Copyright:
© 2019 by The American Society of Hematology.
PY - 2019/9/26
Y1 - 2019/9/26
N2 - 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 5 .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 <10% in 7.7%. Stroke after recovery from acute TTP occurred in 0% (0 of 22) of patients with normal remission ADAMTS13 activity (>70%) and in 27.6% (8 of 29) of patients with low ADAMTS13 activity (£70%; P 5 .007). In conclusion, stroke is common after recovery from TTP and is associated with reduced ADAMTS13 activity during remission.
AB - 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 5 .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 <10% in 7.7%. Stroke after recovery from acute TTP occurred in 0% (0 of 22) of patients with normal remission ADAMTS13 activity (>70%) and in 27.6% (8 of 29) of patients with low ADAMTS13 activity (£70%; P 5 .007). In conclusion, stroke is common after recovery from TTP and is associated with reduced ADAMTS13 activity during remission.
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U2 - 10.1182/blood.2019001056
DO - 10.1182/blood.2019001056
M3 - Article
C2 - 31431443
AN - SCOPUS:85072662137
SN - 0006-4971
VL - 134
SP - 1037
EP - 1045
JO - Blood
JF - Blood
IS - 13
ER -