Recombinant ADAMTS13 for Immune Thrombotic Thrombocytopenic Purpura.
N Engl J Med
; 390(18): 1690-1698, 2024 May 09.
Article
in En
| MEDLINE
| ID: mdl-38718359
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.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Purpura, Thrombotic Thrombocytopenic
/
ADAMTS13 Protein
Limits:
Adult
/
Female
/
Humans
Language:
En
Journal:
N Engl J Med
/
N. Engl. j. med
/
New England journal of medicine
Year:
2024
Document type:
Article
Country of publication: