Nathalie Itzhar-Baïkian

Evaluation of a semi-automated test for quantification of von Willebrand multimers

Christophe Peronino, Nadia Rivet, Nathalie Itzhar-Baïkian, Aurélien Philippe et autres

The assessment of von Willebrand factor (VWF) multimer distribution, particularly following the implantation of circulatory support devices, is a crucial parameter in hemostasis. Our study aimed to evaluate the semi-automated quantification of VWF multimers using the Sebia Hydrasys analyzer. Our analysis focused …

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Relapse of acquired von Willebrand syndrome in a patient non-compliant with Crohn's disease

Myriam Hormi, Melchior Le Mene, Céline Comparon, Imededdine El Kout et autres

We report a case of acquired von Willebrand syndrome relapse in association with Crohn's disease, in a context of non-compliance in a 85-year-old woman suffering from epistaxis and melena. The acquired von Willebrand syndrome is a rare bleeding disorder. This case underlines …

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Acquired hemophilia A: clinical and biological characteristics and therapeutic management of a series of eight patients hospitalized in Lariboisière and Saint-Louis hospitals

Clara Noizat, Nicolas Béranger, Bérangère S. Joly, Agnès Veyradier et autres

Acquired hemophilia A is a rare autoimmune disease, linked to the appearance of autoantibodies directed against circulating factor VIII, and characterized by a major hemorrhagic syndrome. Acquired hemophilia A is a life-threatening diagnostic and therapeutic medical emergency. We describe here the cohort …

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Pathophysiology and abnormalities of hemostasis in intensive care patients with Covid-19

Bérangère S. Joly, Nicolas Béranger, Maxime Delrue, Marie Neuwirth et autres

L’infection respiratoire causée par le coronavirus 2 (Sars-CoV-2), maintenant nommée Covid-19 (pour coronavirus disease 2019), a été qualifiée de pandémie en 2020. L’infection par le Sars-CoV-2 peut conduire à une réponse immunitaire inadaptée, et en particulier à une amplification de la réponse …

Management of von Willebrand disease with a factor VIII‐poor von Willebrand factor concentrate: Results from a prospective observational post‐marketing study

Jenny Goudemand, Françoise Bridey, Ségolène Claeyssens, Nathalie Itzhar-Baïkian et autres

BACKGROUND: , was approved in France in 2003, and then in other countries for the treatment of patients with von Willebrand disease (VWD). OBJECTIVE: To investigate long-term safety and efficacy of the product in real-life over the first 5 post-approval years. PATIENTS/METHODS: …

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Updated overview on von Willebrand disease: focus on the interest of genotyping

Nathalie Itzhar-Baïkian, Pierre Boisseau, Bérangère S. Joly, Agnès Veyradier

Introduction: Von Willebrand disease (VWD) is the most common inherited bleeding disorder, characterized by a quantitative or qualitative defect of von Willebrand factor (VWF), a multimeric glycoprotein crucial for primary hemostasis and coagulation. VWD pathophysiology is heterogeneous as it includes several types …

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[Von Willebrand disease in the elderly].

Nathalie Itzhar-Baïkian, Agnès Veyradier, Jean‐Guillaume Dillinger, Alain Stépanian et autres

Von Willebrand disease in the elderly. Von Willebrand disease (VWD) is a rare inherited haemorrhagic disorder, the prevalence of symptomatic individuals is around 1/10 000. Von Willebrand factor level increases with advanced age, explaining a lower frequency and a lower severity of …

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Differences in von Willebrand factor function in type 2A von Willebrand disease and left ventricular assist device‐induced acquired von Willebrand syndrome

Shannen J. Deconinck, Claudia Tersteeg, Els Bailleul, Leen Delrue et autres

BACKGROUND: Patients with von Willebrand disease (VWD) type 2A or acquired von Willebrand syndrome (aVWS) as a consequence of implantation of left ventricular assist devices (LVAD) are both characterized by a loss of von Willebrand factor (VWF) function. Loss of VWF function …

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Deep venous thrombosis treated by rivaroxaban in a young patient with type Ia carbohydrate-deficient glycoprotein (CDG) syndrome

Bertrand Lefrère, Alain Stépanian, Nathalie Itzhar-Baïkian, Perrine Charles et autres

Congenital disorders of glycosylation (CDG) are rare inborn diseases of glycan component of N-glycosylated proteins. We report here the case of a 28-year-old patient with CDG syndrome type Ia, who presented with a deep venous thrombosis in the left suro-popliteal vein with …

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P3277Distinct differences in laboratory findings of patients with von Willebrand disease type 2A versus patients with LVAD-induced acquired von Willebrand syndrome

Shannen J. Deconinck, Claudia Tersteeg, Els Bailleul, Leen Delrue et autres

Background/Introduction: Patients suffering from the bleeding disorder von Willebrand disease (VWD) type 2A are diagnosed by severely reduced ratios of VWF:CB/VWF:Ag (<0.7) and VWF:RCo/VWF:Ag (<0.7) and severely reduced high molecular weight (HMW) VWF multimers (0–15%). Patients with implanted left ventricular assist devices …

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A Laboratory Phenotype/Genotype Correlation of 1167 French Patients From 670 Families With von Willebrand Disease

Agnès Veyradier, Pierre Boisseau, Édith Fressinaud, Claudine Caron et autres

von Willebrand disease (VWD) is a genetic bleeding disease due to a defect of von Willebrand factor (VWF), a glycoprotein crucial for platelet adhesion to the subendothelium after vascular injury. VWD include quantitative defects of VWF, either partial (type 1 with VWF …

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Combined factor V and VIII deficiency and pregnancy – Need for an early protocol-based multidisciplinary management

Clément Hoffmann, Elisabeth Falzone, Andrada Doina Mihai, Laurence Gitz et autres

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