Thrombotic thrombocytopenic purpura: celebrating 25 years of ADAMTS13 - PubMed
4 hours ago
- #thrombotic disorders
- #ADAMTS13
- #TTP
- Thrombotic Thrombocytopenic Purpura (TTP) was first described over a century ago.
- ADAMTS13, identified 25 years ago, is the enzyme deficient in both immune and congenital TTP.
- Discovery of ADAMTS13 has significantly improved TTP outcomes.
- Understanding ADAMTS13's interaction with platelets and vWF led to quicker diagnosis and novel therapies.
- Key treatments include rituximab (anti-CD20) and caplacizumab for immune TTP, and recombinant ADAMTS13 for congenital TTP.
- Monitoring ADAMTS13 activity helps tailor treatments for both immune and congenital TTP.
- Future developments may include expanded use of recombinant ADAMTS13, quicker assays, and new therapies.
- Potential roles for ADAMTS13 in other thrombotic conditions are being explored.