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Largest comparison between onset and relapses of acquired thrombotic thrombocytopenic purpura reveals severe neurological involvement and worse analytic parameters at debut

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Abstract

It has been proposed that the onset of Acquired Thrombotic Thrombocytopenic Purpura (iTTP) is more severe than subsequent relapses; however, existing studies have limitations. We conducted a retrospective observational study to compare analytical and clinical severity of onset and relapse aTTP cases between 2012 and 2023. A total of 370 episodes of aTTP were analyzed, comprising 272 at initial diagnosis and 98 relapses. At onset, analytical parameters indicative of severity (low hemoglobin, low platelet count, and increased LDH) were significantly worse; patients had severe neurological symptoms (p<0.001) and ≥ 3 points in the TMA mortality score (p<0.001). In conclusion, the onset of aTTP is associated with worse analytical parameters and severe neurological involvement.

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Authors and Affiliations

Authors

Contributions

BVV and RCE contributed equally to the design of the study, data collection, statistical analysis, writing, and critical revision of the final manuscript being both first authors. SMM, MCS, IAM, MMP, BSL, YRR, JCM, PEM, PRG, SFB, MPMH, FAGF, MGA, AVM, CBC, and JMN contributed to the data collection, sample analysis, and critical revision of the final manuscript. Also, all authors contributed to a literature review on the topic.

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Correspondence to Rodrigo Cantera Estefanía.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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Vegas Villalmanzo, B., Cantera Estefanía, R., Muñoz Madrid, S. et al. Largest comparison between onset and relapses of acquired thrombotic thrombocytopenic purpura reveals severe neurological involvement and worse analytic parameters at debut. Ann Hematol 103, 725–727 (2024). https://doi.org/10.1007/s00277-024-05634-x

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