AI Article Synopsis
- - Severe ADAMTS13 deficiency (<10 iu/dL) is a key indicator of thrombotic thrombocytopenic purpura (TTP), leading to serious health issues like microthrombi and platelet aggregation.
- - The article explores the consequences of low ADAMTS13 levels not only during acute TTP episodes but also in patients during clinical remission, indicating ongoing risks.
- - Research indicates that low ADAMTS13 activity may also affect vascular health in diseases beyond TTP, suggesting potential benefits from recombinant ADAMTS13 therapy in treating other thrombotic conditions.
Article Abstract
Severe deficiency of ADAMTS13 (<10 iu/dL) is diagnostic of thrombotic thrombocytopenic purpura (TTP) and leads to accumulation of ultra-large vWF multimers, platelet aggregation, and widespread microthrombi, which can be life-threatening. However, the clinical implications of a low ADAMTS13 activity level are not only important in an acute episode of TTP. In this article, we discuss the effects of low ADAMTS13 activity in congenital and immune-mediated TTP patients not only at presentation but once in a clinical remission. Evidence is emerging of the clinical effects of low ADAMTS13 activity in other disease areas outside of TTP, and here, we explore the wider impact of low ADAMTS13 activity on the vascular endothelium and the potential for recombinant ADAMTS13 therapy in other thrombotic disease states.
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Source |
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11396319 | PMC |
| http://dx.doi.org/10.3390/jcm13175152 | DOI Listing |
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