Publications by authors named "Antoine Babuty"
Introduction: Lupus-anticoagulant hypoprothrombinemia syndrome (LAHS) is a rare but potentially serious condition. LAHS can be of post-infectious (PI) or autoimmune (AI) origin. However, there is currently no clear data available on the differences between these two forms.
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- - Infusion of von Willebrand factor (VWF) and factor VIII (FVIII) can lead to the development of anti-VWF antibodies, complicating management of bleeding in patients with type 3 von Willebrand disease.
- - A systematic review identified 15 cases where patient management varied, with treatments including continuous FVIII infusion, recombinant FVIIa, and other strategies.
- - Emicizumab, a bispecific monoclonal antibody, was used off-label in three cases and proved effective in managing a complex local case, highlighting its potential as a treatment option for patients with allo-immunisation.
Introduction: Acquired von Willebrand syndrome (AVWS) is a rare haemorrhagic disorder. The prophylaxis and treatment of bleeding before surgery are complex. Since 2018, a new recombinant VWF (rVWF) concentrate that contains no factor VIII (FVIII) but a high amount of high molecular weight VWF multimers has been available in France.
View Article and Find Full Text PDFIntroduction: The entity entitled bleeding disorder of unknown cause (BDUC) qualifies individuals displaying a mild haemorrhagic profile but normal routine coagulation tests. This study was designed to evaluate whether collagen-binding assay for von Willebrand Factor (VWF) measurement (VWF:CB) could allow to diagnose VW disease in such patients.
Methods: A large screening was conducted prospectively in two University Hospitals, using the bleeding assessment tool (BAT) recommended by the International Society of Thrombosis and Hemostasis.
Article Synopsis
- Type 2 Normandy von Willebrand disease (VWD2N) is generally seen as a mild bleeding disorder treatable with desmopressin (DDAVP), but the genetic variations among patients, especially the common variant p.Arg854Gln (R854Q), can significantly influence disease severity and treatment response.
- A study involving 123 VWD2N patients analyzed their phenotype and DDAVP response based on genotype, revealing that those with R854Q alleles exhibited different clinical outcomes and bleeding symptoms compared to those without.
- The findings indicate that genetic factors, particularly the presence of the R854Q variant, affect factor VIII levels and the efficacy of DDAVP, highlighting the importance of tailored treatment approaches for V
Article Synopsis
- Von Willebrand disease (VWD) is the most common inherited bleeding disorder, but data on how patients are treated during hospitalizations is limited.
- A study named Hopscotch-WILL looked at 988 VWD patients over four years to understand their treatment and factors driving the use of coagulation factors during hospital stays.
- Results showed that 15% of patients were hospitalized needing von Willebrand factor treatment, with low levels of VWF and FVIII being significant, and usage varied based on VWD type and the reason for hospitalization.
Article Synopsis
- Turoctocog alfa, a Factor VIII replacement therapy, was evaluated for its effectiveness in patients with hemophilia A undergoing surgery, using data from a national database.
- The study analyzed 86 surgeries (49 major and 37 minor) across 56 patients, revealing a high overall clinical efficacy of 89.5%, with minimal complications.
- Results showed that patients with mild hemophilia required fewer infusions and lower doses than those with severe hemophilia, and no significant adverse events or complications occurred during the procedures.
Background: The bleeding risk of patients with mild platelet function disorders is difficult to assess and their phenotype remains ill-explored.
Aim: This study was designed to establish a comprehensive biological phenotype of patients with mild platelet function disorders.
Methods: Twenty patients were included with persistent abnormal light transmission aggregometry (LTA).
Constitutional thrombocytopenias are rare disorders, often difficult to discriminate from acquired thrombocytopenias. More than 80 genes have been described as being at the origin of these diseases. Among them, several variants of the glycoprotein Ib platelet subunit alpha (GP1BA) and glycoprotein Ib platelet subunit beta (GP1BB) genes, coding for the GpIb-IX-V glycoprotein complex, have been reported in the literature.
View Article and Find Full Text PDFHemophilia A is an inherited X-linked recessive bleeding disorder caused by deficient activity of blood coagulation factor VIII (FVIII). In addition, hemophilia patients show associated diseases including osteopenia, altered inflammation and vascular fragility which may represent the consequence of recurrent bleeding or may be related to the direct FVIII deficiency. Nowadays, recombinant FVIII is proposed to treat hemophilia patients with no circulating FVIII inhibitor.
View Article and Find Full Text PDFImmune thrombocytopenia (ITP) is an acquired bleeding disorder, for which no specific diagnostic test exists. Inherited thrombocytopenia (IT) can mimic ITP and lead to unappropriated management with significant morbidity. Here, in small cohorts of these two disorders, we explored whether platelet sialylation and platelet activation could allow to discriminate the two conditions.
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- * The deficiency in FVIII is linked to issues like abnormal bone remodeling and inflammation, which can create unusual blood vessel growth.
- * New treatments, like emicizumab, aim to improve blood clotting by bypassing the antibodies that stop FVIII infusions from working, and there is ongoing research into FVIII's additional roles for future therapies.
Background: Despite their usefulness in perioperative and acute care settings, factor-Xa inhibitor-specific assays are scarcely available, contrary to heparin anti-Xa assay. We assessed whether the heparin anti-Xa assay can (1) be used as a screening test to rule out apixaban, rivaroxaban, fondaparinux, and danaparoid levels that contraindicate invasive procedures according to current guidelines (>30 ng·mL-1, >30 ng·mL-1, >0.1 µg·mL-1, and >0.
View Article and Find Full Text PDFPlatelet immunophenotyping in health and inherited bleeding disorders, a review and practical hints.
Cytometry B Clin Cytom
November 2020
Inherited platelet function disorders are rare hemorrhagic diseases. The gold standard for their exploration is optical aggregometry; however, investigations by flow cytometry (FCM) are being increasingly used. In this review, the physiology of platelets is first recalled, setting the stage for the compartments of platelets that can be apprehended by specific and appropriate labeling.
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