Abstract | INTRODUCTION: MATERIAL AND METHODS: We gathered clinical and biological data from a cohort of 114 patients with acquired TTP at acute phase. VWF-HMWM were assessed by electrophoretic analysis and by an ELISA measuring the capacity of VWF to bind to collagen (VWF:CB), and linear correlation between these two methods was carried out. We cross-referenced clinical and biological data with VWF-HMWM levels. RESULTS: VWF-HMWM levels were heterogeneous, but half of our patients were below normal range (50% if assessed by electrophoresis; 47.4% if assessed by ELISA). The correlation study between electrophoresis and ELISA reached statistical significance (r2 = 0.5979; p < 0.0001). Statistical analysis showed that loss of VWF-HMWM as assessed by VWF:CB < 70 IU/dL is associated with detectable anti-ADAMTS13 antibodies, severe neurological symptoms and thrombocytopenia (p < 0.05). CONCLUSION: Our results confirm that VWF-HMWM can be satisfactorily assessed by VWF:CB, much easier to perform than electrophoresis. The association highlighted between loss of VWF-HMWM, detectable anti-ADAMTS13 IgG and neurological symptoms may offer new insights to understanding the pathophysiology of acquired auto-immune TTP.
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Authors | Nicolas Béranger, Sandrine Benghezal, Sylvaine Savigny, Sophie Capdenat, Bérangère S Joly, Paul Coppo, Alain Stepanian, Agnès Veyradier |
Journal | Thrombosis research
(Thromb Res)
Vol. 181
Pg. 29-35
(Sep 2019)
ISSN: 1879-2472 [Electronic] United States |
PMID | 31330376
(Publication Type: Journal Article)
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Copyright | Copyright © 2019 Elsevier Ltd. All rights reserved. |
Chemical References |
- Immunoglobulin G
- von Willebrand Factor
- ADAMTS13 Protein
- ADAMTS13 protein, human
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Topics |
- ADAMTS13 Protein
(metabolism)
- Adolescent
- Adult
- Aged
- Child
- Child, Preschool
- Female
- Humans
- Immunoglobulin G
(metabolism)
- Male
- Middle Aged
- Nervous System Diseases
(etiology, genetics, pathology)
- Purpura, Thrombotic Thrombocytopenic
(complications, genetics, pathology)
- Young Adult
- von Willebrand Factor
(metabolism)
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