A5022435450- Hemophilia Treatment and Research
- Platelet Disorders and Treatments
- Blood Coagulation and Thrombosis Mechanisms
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Myeloproliferative Neoplasms: Diagnosis and Treatment
- Chronic Myeloid Leukemia Treatments
- Blood groups and transfusion
- Cancer-related gene regulation
- Hemostasis and retained surgical items
- Erythrocyte Function and Pathophysiology
- Cell Adhesion Molecules Research
- Cellular Mechanics and Interactions
- Microfluidic and Bio-sensing Technologies
- Blood disorders and treatments
- Heparin-Induced Thrombocytopenia and Thrombosis
- Blood properties and coagulation
- Thyroid Cancer Diagnosis and Treatment
- Immunodeficiency and Autoimmune Disorders
- Parvovirus B19 Infection Studies
- Multiple Myeloma Research and Treatments
- Blood donation and transfusion practices
- Heme Oxygenase-1 and Carbon Monoxide
- Biomedical Text Mining and Ontologies
- Endodontics and Root Canal Treatments
- Adolescent and Pediatric Healthcare
Inserm
2016-2025
Université de Rennes
2019-2025
Institut de Recherche en Santé, Environnement et Travail
2016-2025
Centre Hospitalier Universitaire de Rennes
2016-2025
École des Hautes Études en Santé Publique
2019-2025
Hôpital Pontchaillou
2007-2021
Hôpital Universitaire Dupuytren
2009
Assistance Publique – Hôpitaux de Paris
1999-2007
Bicêtre Hospital
1999-2007
Hôpital de la Timone
2007
Summary Although extremely rare, acquired haemophilia A (AHA) can cause severe bleeding, which may be fatal. The underlying causes of autoantibody development are not fully understood. Treatment goals bleeding control and eradication. At the time our study, there was no consensus on a standard treatment strategy for AHA. Previous data were mainly retrospective or from single‐centre cohorts. We conducted prospective, controlled, registry‐based study patients with AHA in France. prospective...
BackgroundAcquired hemophilia A (AHA) is a rare autoimmune disorder due to autoantibodies against Factor VIII, with high mortality risk. Treatments aim control bleeding and eradicate antibodies by immunosuppression. International recommendations rely on registers international expert panels.MethodsCREHA, an open-label randomized trial, compared the efficacy safety of cyclophosphamide rituximab in association steroids patients newly diagnosed AHA. Participants were treated 1 mg/kg prednisone...
H. Brouk1,*; A. Amireche1; Kessira1 1Hemobiology and blood transfusion center, faculty of medicine, University Badji Mokhtar Annaba, Algeria Introduction: Combined deficiency coagulation factors V VIII (F5F8D) is a constitutional bleeding disorder, described since 1954 by Oeri. It rare, autosomal recessive, with an estimated prevalence 1/100,000 to 1/1,000,000, representing the most frequent form abnormality, associating more than one factor, not related accidental coincidence several...
Lysophosphatidic acid (LPA) and sphingosine 1-phosphate (S1P) are structurally related bioactive lipids with growth factor-like activities. LPA S1P naturally produced in vivo by blood platelets upon platelet aggregation at least vitro fibroblasts, adipocytes, multiple types of tumor cells. Breast cancer cells respond to S1P. However, their specific actions on breast cell biological functions remain unclear. We therefore conducted an side-by-side study these two mediates human MDA-BO2...
Abstract Introduction FVIII inhibitor development is the most serious contemporary treatment complication in haemophilia A, particularly previously untreated patients (PUPs). No inhibitors developed clinical trials treated with simoctocog alfa (Nuwiq), a fourth-generation recombinant produced human cell line. Methods The NuProtect study investigated immunogenicity of PUPs. was prospective, multinational, open-label, non-controlled, phase III study. PUPs severe A (FVIII:C <1%) any age and...
Background: Real-world data assessing treatment outcomes in patients with hemophilia A routine clinical practice are limited. Objective: To evaluate the effectiveness and safety of octocog alfa moderate/severe receiving practice. Design: The international Antihemophilic Factor Hemophilia Outcome Database study is an observational, noninterventional, prospective, multicenter study. Methods: This planned interim read-out was conducted following 7 years observation (cut-off, 30 June 2020)....
Induction of heme oxygenase-1, a stress-inducible enzyme with anti-inflammatory activity, reduces the immunogenicity therapeutic factor VIII in experimental hemophilia A. In humans, oxygenase-1 expression is modulated by polymorphisms promoter oxygenase-1-encoding gene (HMOX1). We investigated relationship between HMOX1 and inhibitor development severe performed case-control study on 99 inhibitor-positive patients 263 who did not develop inhibitors within first 150 cumulative days exposure...
Abstract Cardiovascular diseases (CVDs) are a major issue in aging patients with hemophilia (PWHs). Antithrombotic agents widely used the general population for CVD treatment, but this recommendation is not fully applicable to PWHs. To improve treatment strategies, prospective case–control study (COCHE) that analyzed management and follow-up (2 years/patient) PWHs was performed France from 2011 2018. In total, 68 (median age: 65 years [39–89]; 48 mild, 10 moderate, severe hemophilia) were...
Introduction Nuwiq ® (Human‐cl rh FVIII ) is a fourth generation recombinant , produced in human cell line, without chemical modification or protein fusion. No inhibitors developed studies with 201 previously treated patients haemophilia A ( HA ). The immunogenicity, efficacy and safety of untreated PUP s) severe are being assessed the ongoing NuProtect study. Methods study, conducted across 38 centres worldwide, evaluating 110 true s all ages ethnicities enrolled for study up to 100...
Hemophilia A (HA) is an X-linked hereditary bleeding disorder defined by a qualitative and/or quantitative factor VIII (FVIII) deficiency. The molecular diagnosis of HA challenging because the high number different causative mutations that are distributed throughout large F8 gene. putative role novel mutations, especially missense may be difficult to interpret as causing HA. We identified 95 out 180 responsible for in 515 patients from 406 unrelated families followed up at single hemophilia...
Summary. Type I Glanzmann's thrombasthenia is a rare congenital platelet function disorder, characterized by undetectable membrane glycoprotein IIb–IIIa (GPIIb–IIIa). Severe bleeding controlled transfusion of normal platelets, leading in some cases to the occurrence anti‐GPIIb–IIIa isoantibodies, which induces loss transfused efficacy. We used immunoadsorption on protein A Sepharose (IA‐PA), has been shown be efficient decreasing titre antibodies several immune diseases, three patients with...
Summary Recombinant activated factor VII (rFVIIa) is an effective haemostatic treatment in haemophiliacs with inhibitors. In vitro, FVIIa concentrations corresponding to those obtained therapeutic doses of rFVIIa have been shown induce normal thrombin generation and platelet activation the absence factors VIII or IX. To further study vivo changes induced by rFVIIa, circulating procoagulant microparticles (MP) were measured patients treated discontinuous injections Novoseven®. 6 out 15...
Introduction:During the treatment of Hemophilia A patients without inhibitors using factor VIII (FVIII) activity-mimicking bispecific antibody Emicizumab (Hemlibra) bleeding episodes can occur, which require administration FVIII concentrates to restore hemostasis.Because Emicizumab's specificity for human coagulation factors, levels in presence be measured by a chromogenic activity assay based on bovine reagents.However, novel agents could lack this and thus not allow successfully follow...
Acquired haemophilia is a life‐threatening disorder caused by circulating auto‐antibodies that inhibit factor VIII coagulant activity (FBIII:C). Immunoadsorption on protein A sepharose (IA‐PA) was performed in two bleeding patients with acquired haemophilia: we observed dramatic and quick decrease the anti‐FVIII:C inhibitor titre leading to normal, albeit transient, haemostatic status. In one case, IA‐PA only procedure which succeeded stopping massive haemorrhage. second reinforced effect of...
We retrospectively analysed the data files of 171 adults and 87 children/adolescents with severe haemophilia, except for 14 patients (moderate; minor) (1), to develop a global population pharmacokinetic (PK) model eight factors VIII (FVIII) that could estimate individual PK parameters targeting desired level FVIII activity (FVIII:C); (2) compare half-life (HL) in switching from standard (SHL) an extended (EHL) evaluate relevance switch. One-stage clotting assay measurement (FVIII:C, IU/mL)...
Despite the wide use of bleeding scores and reliability clotting factor level measurement, risk stratification before surgery remains challenging in patients with rare inherited disorders.This multicenter observational prospective study assessed coagulation deficiency, perioperative hemostatic management choices by hemostasis experts outcomes after surgery.One hundred seventy-eight low activity (factor [F] II, FV, combined FV-FVIII, FVII, FX, or FXI <50%) underwent 207 surgical procedures....
Platelet and cell stimulation lead to plasma membrane remodelling, resulting in phosphatidylserine (PS) externalisation the outer leaflet of membrane, associated with shedding PS-rich microparticles (MPs), contributes thrombin generation by promoting assembly coagulation enzyme complexes. A previous study assessed MP levels haemophiliacs after haemostatic treatment. To further investigate vivo characteristics were studied 79 62 non-haemophiliacs. Both groups showed heterogeneity levels,...