Document Type
Article
Publication Date
3-11-2024
Abstract
Thrombotic thrombocytopenic purpura (TTP) is an uncommon, but potentially disabling or even deadly, thrombotic microangiopathy with a well-studied mechanism of ADAMTS13 deficiency or dysfunction. While established treatments are largely effective, the standard ADAMTS13 testing required to definitively diagnose TTP may cause delays in diagnosis and treatment, highlighting the need for rapid and effective diagnostic methods. Additionally, the heterogeneous presentation and varied inciting events of TTP suggest more variation in its mechanism than previously thought, implying three potential pathways rather than the accepted two. The recent discovery of ADAMTS13 conformation as a potential contributor to TTP in addition to the proposal of using the absolute immature platelet count (A-IPC) as a biomarker, present novel areas for monitoring and treatment. A-IPC in particular may serve as a more rapid and accurate diagnostic test to distinguish TTP from non-TTP TMAs and to monitor treatment response and relapse. These considerations highlight the need to further study TTP in order to improve best practices and patient care.
Keywords
autoimmunity, coagulation, immunity, platelets, thrombotic thrombocytopenic purpura
Language
English
Publication Title
Biomedicines
Rights
© 2024 by the authors. Licensee MDPI, Basel, Switzerland. This is an Open Access work distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0/) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 International License.
Recommended Citation
Dolin, H. H., & Maitta, R. W. (2024). Pathological Mechanisms and Novel Testing Methods in Thrombotic Thrombocytopenic Purpura. Biomedicines, 12(3), 621. https://doi.org/10.3390/biomedicines12030621
Manuscript Version
Final Publisher Version