Article: Is Endothelial Activation a Critical Event in Thrombotic Thrombocytopenic Purpura?
2023 Volume 12, Issue 3
Abstract: Thrombotic thrombocytopenic purpura (TTP) is a severe thrombotic microangiopathy. The current pathophysiologic paradigm suggests that the ADAMTS13 deficiency leads to Ultra Large-Von Willebrand Factor multimers accumulation with generation of ... ...
| Abstract | Thrombotic thrombocytopenic purpura (TTP) is a severe thrombotic microangiopathy. The current pathophysiologic paradigm suggests that the ADAMTS13 deficiency leads to Ultra Large-Von Willebrand Factor multimers accumulation with generation of disseminated microthrombi. Nevertheless, the role of endothelial cells in this pathology remains an issue. In this review, we discuss the various clinical, in vitro and in vivo experimental data that support the important role of the endothelium in this pathology, suggesting that ADAMTS13 deficiency may be a necessary but not sufficient condition to induce TTP. The "second hit" model suggests that in TTP, in addition to ADAMTS13 deficiency, endogenous or exogenous factors induce endothelial activation affecting mainly microvascular cells. This leads to Weibel-Palade bodies degranulation, resulting in UL-VWF accumulation in microcirculation. This endothelial activation seems to be worsened by various amplification loops, such as the complement system, nucleosomes and free heme. |
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| Language | English |
| Publishing date | 2023-01-18 |
| Publishing country | Switzerland |
| Document type | Journal Article ; Review |
| ZDB-ID | 2662592-1 |
| ISSN | 2077-0383 |
| ISSN | 2077-0383 |
| DOI | 10.3390/jcm12030758 |
| Database | MEDical Literature Analysis and Retrieval System OnLINE |
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