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Roose, Elien; Schelpe, An-Sofie; Tellier, Edwige; Sinkovits, György; Joly, Bérangère S.; Dekimpe, Charlotte; Kaplanski, Gilles; Le Besnerais, Maelle; Mancini, Ilaria; Falter, Tanja; Von Auer, Charis; Feys, Hendrik B.; Reti, Marienn; Rossmann, Heidi; Vandenbulcke, Aline; Pareyn, Inge; Voorberg, Jan; Greinacher, Andreas; Benhamou, Ygal; Deckmyn, Hans; Fijnheer, Rob; Prohászka, Zoltan; Peyvandi, Flora; Lämmle, Bernhard; Coppo, Paul; De Meyer, Simon F.; Veyradier, Agnès; Vanhoorelbeke, Karen
Blood, 07/2020, Letnik: 136, Številka: 3Journal Article
Recently, we showed that ADAMTS13 circulates in an open conformation during the acute phase of immune-mediated thrombotic thrombocytopenic purpura (iTTP). Although the cause of this conformational change remains elusive, ADAMTS13 is primarily closed in iTTP patients in remission with ADAMTS13 activity >50% and undetectable anti-ADAMTS13 autoantibodies, as well as after rituximab treatment, suggesting a role for anti-ADAMTS13 autoantibodies. Therefore, immunoglobulin G from 18 acute iTTP patients was purified and added to closed ADAMTS13 in healthy donor plasma. This resulted in open ADAMTS13 in 14 of 18 (78%) samples, proving that 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 from iTTP patients in remission in whom ADAMTS13 activity was <50%. The open ADAMTS13 conformation was found during acute iTTP, as well as in patients in remission with ADAMTS13 activity <50% and in half of the patients with ADAMTS13 activity >50%, although free anti-ADAMTS13 autoantibodies were not always detected. Thus, open ADAMTS13 is a hallmark of acute iTTP, as well as a novel biomarker that can be used to detect subclinical iTTP in patients in remission. Finally, a long-term follow-up study in 1 iTTP patient showed that the open conformation precedes a substantial 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. •Anti-ADAMTS13 autoantibodies from iTTP patients induce an open ADAMTS13 conformation.•An open ADAMTS13 conformation is a novel and sensitive biomarker for subclinical iTTP. Display omitted
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JCR | SNIP | JCR | SNIP | JCR | SNIP | JCR | SNIP |
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in: SICRIS
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