Long‐term cognitive impairments following recovery in the patients with immune‐mediated thrombotic thrombocytopenic purpura

2020 ◽  
Vol 191 (5) ◽  
pp. 671-672
Author(s):  
Kazuya Sakai
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Cognitive Impairments ◽  
Thrombocytopenic Purpura ◽  
Immune Mediated

scholarly journals Open ADAMTS13, induced by antibodies, is a biomarker for subclinical immune-mediated thrombotic thrombocytopenic purpura

Blood ◽  
2020 ◽  
Author(s):  
Elien Roose ◽  
An-Sofie Schelpe ◽  
Edwige Tellier ◽  
György Sinkovits ◽  
Bérangère S Joly ◽  
...  
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Adamts13 Activity ◽  
Thrombocytopenic Purpura ◽  
Follow Up Study ◽  
Open Conformation ◽  
Immune Mediated ◽  
Long Term Follow Up ◽  
Donor Plasma

Recently, we showed that during the acute phase of immune-mediated thrombotic thrombocytopenic purpura (iTTP), ADAMTS13 circulates in an open conformation. Although the cause of this conformational change in acute iTTP remains elusive, ADAMTS13 is mainly closed in iTTP patients (i) in remission with an ADAMTS13 activity >50% and undetectable anti-ADAMTS13 autoantibodies, and (ii) after rituximab treatment, suggesting a role for anti-ADAMTS13 autoantibodies. Therefore, IgGs from 18 acute iTTP patients were purified and added to closed ADAMTS13 in healthy donor plasma. This resulted in open ADAMTS13 in 14/18 (78%) samples, proving that indeed anti-ADAMTS13 autoantibodies can induce an open ADAMTS13 conformation. To further elucidate the conformation of ADAMTS13 in iTTP patients, we studied a novel iTTP patient cohort (n=197) that also included plasma samples of iTTP patients in remission where ADAMTS13 activity was <50%. The open ADAMTS13 conformation was not only found during acute iTTP but also in patients in remission with an ADAMTS13 activity <50% and in half of the patients with an ADAMTS13 activity >50%, although free anti-ADAMTS13 autoantibodies were not always detected. Thus open ADAMTS13 is not only a hallmark of acute iTTP, but also a novel biomarker to detect subclinical iTTP in patients in remission. Finally, a long term follow-up study in one iTTP patient showed that the open conformation precedes a severe drop in ADAMTS13 activity. In conclusion, we have shown that anti-ADAMTS13 autoantibodies from iTTP patients induce an open ADAMTS13 conformation. Most importantly, an open ADAMTS13 conformation is a biomarker for subclinical iTTP and could become an important tool in TTP management.

scholarly journals Preemptive rituximab prevents long-term relapses in immune-mediated thrombotic thrombocytopenic purpura

Blood ◽  
2018 ◽  
Vol 132 (20) ◽  
pp. 2143-2153 ◽  
Author(s):  
Matthieu Jestin ◽  
Ygal Benhamou ◽  
An-Sofie Schelpe ◽  
Elien Roose ◽  
François Provôt ◽  
...  
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Relapse Rate ◽  
Thrombocytopenic Purpura ◽  
Immune Mediated ◽  
Key Points ◽  
Long Term Follow Up ◽  
Adamts13 Deficiency ◽  
Risk Balance

Key Points TTP patients who display persistent and severe ADAMTS13 deficiency after remission have a relapse rate of 74% during long-term follow-up. Preemptive rituximab can decrease TTP relapses in 85% of patients with a favorable benefit-risk balance.

scholarly journals Long-term Kidney Outcomes in Patients With Acquired Thrombotic Thrombocytopenic Purpura

2017 ◽  
Vol 2 (6) ◽  
pp. 1088-1095 ◽  
Author(s):  
Dustin J. Little ◽  
Lauren M. Mathias ◽  
Evaren E. Page ◽  
Johanna A. Kremer Hovinga ◽  
Sara K. Vesely ◽  
...  
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Thrombocytopenic Purpura

Dasatinib-induced immune mediated-thrombotic thrombocytopenic purpura

2018 ◽  
Vol 57 (2) ◽  
pp. 222-224 ◽  
Author(s):  
Esra Terzi Demirsoy ◽  
Ozgur Mehtap ◽  
Elif Birtas Atesoglu ◽  
Pinar Tarkun ◽  
Necmi Eren ◽  
...  
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Thrombocytopenic Purpura ◽  
Immune Mediated
Sign in / Sign up

Export Citation Format

Share Document