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Recombinant ADAMTS13 for Immune Thrombotic Thrombocytopenic Purpura.
- Source :
-
The New England journal of medicine [N Engl J Med] 2024 May 09; Vol. 390 (18), pp. 1690-1698. - Publication Year :
- 2024
-
Abstract
- In patients with immune thrombotic thrombocytopenic purpura (iTTP), autoantibodies against the metalloprotease ADAMTS13 lead to catastrophic microvascular thrombosis. However, the potential benefits of recombinant human ADAMTS13 (rADAMTS13) in patients with iTTP remain unknown. Here, we report the clinical use of rADAMTS13, which resulted in the rapid suppression of disease activity and complete recovery in a critically ill patient whose condition had proved to be refractory to all available treatments. We also show that rADAMTS13 causes immune complex formation, which saturates the autoantibody and may promote its clearance. Our data support the role of rADAMTS13 as a novel adjunctive therapy in patients with iTTP.<br /> (Copyright © 2024 Massachusetts Medical Society.)
- Subjects :
- Female
Humans
Antigen-Antibody Complex blood
Antigen-Antibody Complex immunology
Autoantibodies blood
Autoantibodies immunology
Recombinant Proteins immunology
Recombinant Proteins therapeutic use
Adult
Black or African American
Plasma Exchange
Treatment Outcome
ADAMTS13 Protein immunology
ADAMTS13 Protein therapeutic use
Purpura, Thrombotic Thrombocytopenic diagnosis
Purpura, Thrombotic Thrombocytopenic drug therapy
Purpura, Thrombotic Thrombocytopenic immunology
Purpura, Thrombotic Thrombocytopenic therapy
Subjects
Details
- Language :
- English
- ISSN :
- 1533-4406
- Volume :
- 390
- Issue :
- 18
- Database :
- MEDLINE
- Journal :
- The New England journal of medicine
- Publication Type :
- Academic Journal
- Accession number :
- 38718359
- Full Text :
- https://doi.org/10.1056/NEJMoa2402567