Delayed normalisation of ADAMTS13 activity in acute Thrombotic Thrombocytopenic Purpura in the caplacizumab era
| Autor: | Nithya Prasannan, Mari Thomas, Matthew James Stubbs, John-Paul Westwood, Rens de Groot, Deepak Singh, Marie Scully |
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| Rok vydání: | 2023 |
| Předmět: | |
| Zdroj: | Blood. |
| ISSN: | 1528-0020 0006-4971 |
| DOI: | 10.1182/blood.2022018847 |
| Popis: | The benefits of caplacizumab in acute immune mediated TTP (iTTP) are well established. We identified a delay in normalisation of ADAMTS13 activity (>30%) in a subgroup of caplacizumab treated patients which was not evident in the pre-caplacizumab era. Caplacizumab treated patients (n=64) achieved ADAMTS13 activity >30% at median 31 days post PEX, compared to 11.5 days in the non-caplacizumab group (n=50, p=0.0004). 18/64 (28%) caplacizumab treated patients had an ADAMTS13 activity 30% (median 139 days after completing PEX). 18/64 (28%) of patients receiving extended caplacizumab (31-58 days) failed to achieve ADAMTS13 activity >30% at time of stopping caplacizumab compared to 4/47 (8.5%) of historical controls at similar timepoint (30+28 days, p30% within 30+28 days was 6 times more likely in caplacizumab treated patients (OR 6.3, p=0.0006). ADAMTS13 antigen level |
| Databáze: | OpenAIRE |
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