Thrombotic thrombocytopenic purpura
Délégation Paris 7 · Université Paris Cité · +5 more institutions
Abstract
Abstract Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and organ ischemia linked to disseminated microvascular platelet rich-thrombi. TTP is specifically related to a severe deficiency in ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13), the specific von Willebrand factor-cleaving protease. ADAMTS13 deficiency is most frequently acquired via ADAMTS13 autoantibodies, but rarely, it is inherited via mutations of the ADAMTS13 gene. The first acute episode of TTP usually occurs during adulthood, with a predominant anti-ADAMTS13 autoimmune…
Citation impact
- FWCI
- 22.65
- Percentile
- 100%
- References
- 120
Authors
3- BSBérangère S. JolyCorresponding
Délégation Paris 7, Université Paris Cité, Assistance Publique – Hôpitaux de Paris, Laboratoire National de Référence, Hôpital Lariboisière
- PCPaul Coppo
Sorbonne Université, Assistance Publique – Hôpitaux de Paris, Hôpital Saint-Antoine, Laboratoire National de Référence
- AVAgnès Veyradier
Délégation Paris 7, Université Paris Cité, Assistance Publique – Hôpitaux de Paris, Laboratoire National de Référence, Hôpital Lariboisière
Topics & keywords
- ADAMTS13
- Thrombotic thrombocytopenic purpura
- Medicine
- Rituximab
- Thrombotic microangiopathy
- Microangiopathic hemolytic anemia
- Immunology
- Plasmapheresis
- Good health and well-being