A5014845281- Complement system in diseases
- Platelet Disorders and Treatments
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Renal Diseases and Glomerulopathies
- Blood Coagulation and Thrombosis Mechanisms
- COVID-19 Clinical Research Studies
- Venous Thromboembolism Diagnosis and Management
- Blood groups and transfusion
- Trypanosoma species research and implications
- Hemophilia Treatment and Research
- Iron Metabolism and Disorders
- Acute Myeloid Leukemia Research
- Pregnancy and preeclampsia studies
- Long-Term Effects of COVID-19
- Atrial Fibrillation Management and Outcomes
- Autoimmune and Inflammatory Disorders Research
- Immune Cell Function and Interaction
- Monoclonal and Polyclonal Antibodies Research
- Lymphoma Diagnosis and Treatment
- COVID-19 and healthcare impacts
- Mosquito-borne diseases and control
- Myeloproliferative Neoplasms: Diagnosis and Treatment
- Pharmaceutical studies and practices
- Mechanical Circulatory Support Devices
- Parvovirus B19 Infection Studies
Hôpital Lariboisière
2016-2025
Assistance Publique – Hôpitaux de Paris
2016-2025
Université Paris Cité
2016-2025
Centre de Recherche des Cordeliers
2024-2025
Inserm
2014-2025
Sorbonne Université
2019-2025
Hôpital Saint-Antoine
2019-2025
Sorbonne Paris Cité
2019-2024
Institut de recherche Saint-Louis
2019-2024
Centre National de la Recherche Scientifique
2024
In Immune-mediated Thrombotic Thrombocytopenic Purpura (iTTP), patients develop antibodies against ADAMTS13. The majority of exhibit inhibitory anti-spacer antibodies. Non-inhibitory binding to the carboxy-terminal CUB domains have been suggested enhance clearance ADAMTS13 in iTTP. Furthermore, anti-CUB induce an open conformation, which has shown be important biomarker for disease severity and relapse risk. We explored whether introduction N-glycans ADAMST13 can reduce pathogenic...
The anti-Von Willebrand Factor (VWF) nanobody caplacizumab is licensed for adults with immune-mediated thrombotic thrombocytopenic purpura (iTTP) in association therapeutic plasma exchange (TPE) and immunosuppression. However, whether reduces mortality, its optimal timing of initiation, not completely settled. This international, multicenter retrospective cohort study recruited patients from 2018 until 2023 data collection took place January 1st to June 30th the participating centers. One...
BackgroundThe outbreak of chilblain‐like lesions (CLL) during the COVID‐19 pandemic has been reported extensively, potentially related to SARS‐CoV‐2 infection, yet its underlying pathophysiology is unclear.
Congenital thrombotic thrombocytopaenic purpura (TTP) or Upshaw-Schulman syndrome (USS) is a rare, life-threatening, inherited microangiopathy (TMA). USS mostly due to bi-allelic recessive sequence variations of the disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13 (ADAMTS13) gene inducing severe ADAMTS13 deficiency (activity < 10 IU/dL). In healthy individuals, circulates in folded conformation where CUB domains interact spacer domain. The spacer-CUB interaction...
Patients with severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2)- have been reported to develop endothelium injury, an excessive inflammatory response and subsequent marked hypercoagulability, leading a high prevalence of venous thromboembolic events (VTE), up c. 50% in cases.1, 2 von Willebrand factor (VWF) is known as major cause thrombo-inflammation via mechanisms including endothelial activation, enhanced VWF secretion, assembly hyper adhesive multimers, defective cleavage by...
Summary The immunosuppressive treatment of immune‐mediated thrombotic thrombocytopenic purpura (iTTP) in patients with intolerance or refractoriness to the B‐cell depleting monoclonal antibody rituximab remains debated. Daratumumab, a plasma cell‐directed targeting CD38, represents therapeutic option, but data are scarce. French Thrombotic Microangiopathies Reference Center conducted nationwide survey on iTTP treated daratumumab. Nine episodes from seven were identified. Treatment was...
In up to 25% of patients with acquired TTP, anti-ADAMTS13 antibodies are not identified, the mechanism resulting from ADAMTS13 deficiency remains unidentified (uTTP). this study, we provide further insights on clinical presentation and outcome uTTP. baseline undetectable antibodies, usual features iTTP (young age, cerebral involvement, severe thrombocytopenia) no other associated context than a history systemic autoimmune disease or pregnancy, should prompt consider diagnosis iTTP.