8 results on '"Le Cam-Duchez, V."'
Search Results
2. Cyclophosphamide vs rituximab for eradicating inhibitors in acquired hemophilia A: A randomized trial in 108 patients.
- Author
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Lévesque H, Viallard JF, Houivet E, Bonnotte B, Voisin S, Le Cam-Duchez V, Maillot F, Lambert M, Liozon E, Hervier B, Fain O, Guillet B, Schmidt J, Luca LE, Ebbo M, Ferreira-Maldent N, Babuty A, Sailler L, Duffau P, Barbay V, Audia S, Benichou J, Graveleau J, and Benhamou Y
- Subjects
- Humans, Male, Female, Middle Aged, Aged, Immunosuppressive Agents therapeutic use, Adult, Factor VIII therapeutic use, Factor VIII immunology, Aged, 80 and over, Rituximab therapeutic use, Hemophilia A drug therapy, Cyclophosphamide therapeutic use
- Abstract
Background: Acquired hemophilia A (AHA) is a rare autoimmune disorder due to autoantibodies against Factor VIII, with a high mortality risk. Treatments aim to control bleeding and eradicate antibodies by immunosuppression. International recommendations rely on registers and international expert panels., Methods: CREHA, an open-label randomized trial, compared the efficacy and safety of cyclophosphamide and rituximab in association with steroids in patients with newly diagnosed AHA. Participants were treated with 1 mg/kg prednisone daily and randomly assigned to receive either 1.5-2 mg/kg/day cyclophosphamide orally for 6 weeks, or 375 mg/m
2 rituximab once weekly for 4 weeks. The primary endpoint was complete remission over 18 months. Secondary endpoints included time to achieve complete remission, relapse occurrence, mortality, infections and bleeding, and severe adverse events., Results: Recruitment was interrupted because of new treatment recommendations after 108 patients included (58 cyclophosphamide, 50 rituximab). After 18 months, 39 cyclophosphamide patients (67.2 %) and 31 rituximab patients (62.0 %) were in complete remission (OR 1.26; 95 % CI, 0.57 to 2.78). In the poor prognosis group (FVIII < 1 IU/dL, inhibitor titer > 20 BU mL-1 ), significantly more remissions were observed with cyclophosphamide (22 patients, 78.6 %) than with rituximab (12 patients, 48.0 %; p = 0.02). Relapse rates, deaths, severe infections, and bleeding were similar in the 2 groups. In patients with severe infection, cumulative doses of steroids were significantly higher than in patients without infection (p = 0.03)., Conclusion: Cyclophosphamide and rituximab showed similar efficacy and safety. As first line, cyclophosphamide seems preferable, especially in poor prognosis patients, as administered orally and less expensive., Funding: French Ministry of Health., Clinicaltrials: gov number: NCT01808911., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)- Published
- 2024
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3. First observation of inhibitor development against efmoroctocog alfa in France.
- Author
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Chamouni P, Barbay V, Billoir P, Le Cam-Duchez V, Malassigne C, Massy N, and Königs C
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- Factor VIII adverse effects, France, Humans, Male, Middle Aged, Recombinant Fusion Proteins adverse effects, Antibodies, Bispecific administration & dosage, Antibodies, Monoclonal, Humanized administration & dosage, Blood Coagulation Factor Inhibitors blood, Factor VIII administration & dosage, Hemophilia A blood, Hemophilia A drug therapy, Recombinant Fusion Proteins administration & dosage
- Abstract
In patients with severe haemophilia receiving clotting factor concentrates, the risk of immunisation against their usual treatment is still patent and feared. New haemophilia drug treatments with an extended half-life have become available over the past few years. The risk of inhibitor development to these new treatments is unclear. We report the case of a 51-year-old man with severe haemophilia A, who was previously treated with no history of inhibitor development. Soon after a switch in his treatment to efmoroctocog alfa he developed an inhibitor against this recombinant Fc fusion extended half-life FVIII (rFc-FVIII) product. The patient was on an on-demand treatment regimen and was treated for mucosal bleeding. The inhibitor was characterised as type I, with classical epitope mapping. The spontaneous evolution of this inhibitor was favourable, but an anamnestic response led to a switch in his treatment to emicizumab., (Copyright © 2021 Elsevier Ltd. All rights reserved.)
- Published
- 2021
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4. Factor VIII and IX assays for post-infusion monitoring in hemophilia patients: Guidelines from the French BIMHO group (GFHT).
- Author
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Jeanpierre E, Pouplard C, Lasne D, Le Cam Duchez V, Eschwege V, Flaujac C, Galinat H, Harzallah I, Proulle V, Smahi M, Sobas F, Stepina N, Toulon P, Voisin S, Ternisien C, and Nougier C
- Subjects
- Blood Coagulation, Blood Coagulation Tests methods, Clinical Decision-Making, Disease Management, Drug Monitoring, Factor IX administration & dosage, Factor VIII administration & dosage, Hemophilia A diagnosis, Hemophilia B diagnosis, Humans, Recombinant Proteins administration & dosage, Recombinant Proteins pharmacokinetics, Treatment Outcome, Factor IX pharmacokinetics, Factor VIII pharmacokinetics, Hemophilia A blood, Hemophilia A drug therapy, Hemophilia B blood, Hemophilia B drug therapy
- Abstract
Replacement therapy with plasma-derived or recombinant FVIII and FIX (pdFVIII/pdFIX or rFVIII/rFIX) concentrates is the standard of treatment in patients with haemophilia A and B, respectively. Measurement of factor VIII (FVIII:C) or factor IX (FIX:C) levels can be done by one-stage clotting assay (OSA) or chromogenic substrate assay (CSA). The French study group on the Biology of Hemorrhagic Diseases (a collaborative group of the GFHT and MHEMO network) presents a literature review and proposals for the monitoring of FVIII:C and FIX:C levels in treated haemophilia A and B patients, respectively. The use of CSA is recommended for the monitoring of patients treated with pdFVIII or rFVIII including extended half-life (EHL) rFVIII. Except for rFVIII-Fc, great caution is required when measuring FVIII:C levels by OSA in patients substituted by EHL-rFVIII. The OSA is recommended for the monitoring of patients treated with pdFIX or rFIX. Large discordances in the FIX:C levels measured for extended half-life rFIX (EHL-rFIX), depending on the method and reagents used, must lead to great attention when OSA is used for measuring FIX:C levels in patients substituted by EHL-rFIX. Data of most of recent studies, obtained with spiked plasmas, deserve to be confirmed in plasma samples of treated patients., (© 2020 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)
- Published
- 2020
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5. [Factor VIII assays in treated hemophilia A patients].
- Author
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Lasne D, Pouplard C, Nougier C, Eschwege V, Le Cam Duchez V, Proulle V, Smahi M, Harzallah I, Voisin S, Toulon P, Sobas F, Galinat H, Flaujac C, Ternisien C, and Jeanpierre E
- Subjects
- Blood Chemical Analysis methods, Blood Coagulation Tests methods, Hemophilia A therapy, Humans, Prognosis, Factor VIII analysis, Hemophilia A blood, Hemophilia A diagnosis, Monitoring, Physiologic methods
- Abstract
Replacement therapy with plasma-derived or recombinant FVIII (pdFVIII or rFVIII) concentrates is the standard of treatment in patients with hemophilia A. The reference method used for measuring factor VIII (FVIII:C) levels in patients treated by FVIII concentrates is the chromogenic substrate assay (CSA). However, the one-stage clotting assay (OSA) is predominantly used in current clinical practice, but this method depends on the activated partial thromboplastin time (APTT) reagent and the coagulation analyzer used, and wide variations in the measurements of FVIII recovery have been reported with some factor concentrates. The French study group on the biology of hemorrhagic diseases (a collaborative group of the GFHT and MHEMO network) presents a review of the literature and proposals for the monitoring of FVIII:C levels in treated hemophilia A patients. The use of CSA calibrated with a plasma reference tested against the current FVIII WHO (World Health Organization) International Standard is recommended for the monitoring of patients treated with pdFVIII or rFVIII including extended half-life (EHL) rFVIII. OSA are adequate for the monitoring of patients treated with pdFVIII or with most of rFVIII concentrates. However, preliminary comparison with CSA is mandatory before measuring FVIII:C by OSA in patients treated by Refacto AF
® . For rFVIII-EHL, OSA are only acceptable for Elocta®. Great caution is therefore required when measuring FVIII:C levels by OSA in patients substituted by other EHL-rFVIII. Indeed, most of recent studies reported data obtained with spiked plasmas, which deserve to be confirmed on plasma samples collected in treated patients.- Published
- 2019
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6. [Acquired A hemophilia and lymphoproliferative diseases: A literature review].
- Author
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Le Cam-Duchez V
- Subjects
- Humans, Hemophilia A complications, Lymphoproliferative Disorders complications
- Abstract
Acquired A haemophilia is a rare but severe autoimmune disease. It is caused by autoantibodies against the coagulation factor VIII. These autoantibodies could have different consequences: no effect, inhibition of factor VIII or factor VIII activity hydrolyzing. In about half of the cases, no cause is associated with the disorder [idiopathic acquired A haemophilia]. But in remaining 50% of the patients, some circumstances could be associated with acquired hemophilia: post-partum, autoimmune disorders, cancer and sometimes malignant blood disorders. The objective of this article was to review the literature about acquired A hemophilia associated with lymphoproliferative diseases., (Copyright © 2015 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.)
- Published
- 2015
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7. Acquired hemophilia possibly induced by etanercept in a patient with rheumatoid arthritis.
- Author
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Banse C, Benhamou Y, Lequerré T, Le Cam-Duchez V, Lévesque H, and Vittecoq O
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- Antirheumatic Agents therapeutic use, Etanercept therapeutic use, Female, Humans, Middle Aged, Antirheumatic Agents adverse effects, Arthritis, Rheumatoid drug therapy, Etanercept adverse effects, Hemophilia A chemically induced
- Abstract
A 47-year-old woman with rheumatoid arthritis (RA) treated successively with infliximab, abatacept, and etanercept spontaneously developed subcutaneous bruises and a noncompressive hematoma 11 months after starting etanercept therapy (50mg/week). Her prothrombin time was normal but her activated partial thromboplastin time was increased to 2.48 (normal range, 0.85-1.17). She had a circulating anticoagulant (Rosner index, 45; normal,<13) due to an anti-factor VIII antibody in a titer of 45 Bethesda units. Her factor VIII level was less than 1% (normal range, 55-150). The etanercept and leflunomide were stopped and prednisone was given in a daily dosage of 1mg/kg, in combination with rituximab, two 1-g doses at an interval of 2 weeks. After 5 months, persistence of the anti-factor VIII antibody prompted the initiation of azathioprine therapy, 2mg/kg/d. A remission was achieved 9 months after the diagnosis of acquired hemophilia and was sustained at last follow-up after 3 years. This new case of acquired hemophilia in a patient with RA may reflect a simple association or an inducing role of etanercept., (Copyright © 2015 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.)
- Published
- 2015
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8. Outcome of acquired haemophilia in France: the prospective SACHA (Surveillance des Auto antiCorps au cours de l'Hémophilie Acquise) registry.
- Author
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Borg JY, Guillet B, Le Cam-Duchez V, Goudemand J, and Lévesque H
- Subjects
- Adult, Age Distribution, Aged, Aged, 80 and over, Autoantibodies immunology, Cause of Death, Factor VIII immunology, Female, France epidemiology, Hemophilia A complications, Hemophilia A mortality, Hemorrhage complications, Hemorrhage drug therapy, Hemostatics therapeutic use, Humans, Immunosuppressive Agents therapeutic use, Male, Middle Aged, Prospective Studies, Recurrence, Treatment Outcome, Hemophilia A drug therapy, Hemophilia A epidemiology, Registries
- Abstract
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 are bleeding control and autoantibody eradication. At the time of 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 a prospective, controlled, registry-based study of patients with AHA in France. The prospective French registry (Surveillance des Auto antiCorps au cours de l'Hémophilie Acquise [SACHA]) collected data on prevalence, clinical course, disease associations and outcomes for haemostatic treatment and autoantibody eradication in 82 patients with a 1-year follow-up. Similar to earlier studies, the prevalence of AHA was higher in the elderly, with two thirds of patients aged >70 years. Around half of AHA cases were associated with underlying disease, most commonly autoimmune disease and cancer in younger and older patients respectively. Haemostatic treatment was initially administered to 46% of patients. Complete resolution or improvement of initial bleeding occurred in 22/27 (81%) rFVIIa-treated patients and in all six cases receiving pd-aPCC. The majority of patients (94%) received immunosuppressive therapy, with complete remission at 3 months in 61% (36/59) and in 98% (50/51) at 1 year. Overall mortality was 33%: secondary to bleeding in only three patients but to sepsis in 10. Bypassing agents were effective at controlling bleeding in patients with AHA. Immunosuppressive therapy should be used early but with caution, particularly in elderly patients., (© 2013 John Wiley & Sons Ltd.)
- Published
- 2013
- Full Text
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