Life-threatening bleeding in patients with hemophilia (PWH): a 10-year cohort study in Dakar, Senegal

Touré, Sokhna Aïssatou; Seck, Moussa; Sy, Diariatou; Bousso, Elimane Seydi; Faye, Blaise Felix; Diop, S

doi:10.1080/16078454.2022.2047286

Cited by 7 publications

(9 citation statements)

References 20 publications

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“…28−33 More recently, a large retrospective cohort study confirmed that FXI deficiency (FXI activity < 50%) was associated with an increased risk of severe bleeding and clinically relevant nonsevere bleeding, primarily postprocedure. 34 This phenotype is unlike the more frequent spontaneous bleeding found in patients with hemophilia A (factor VIII deficiency) and hemophilia B (factor IX deficiency), 28,35 supporting a minor role of FXI in hemostasis.…”

Section: Hemostasismentioning

confidence: 95%

“…Although the intrinsic pathway amplifies clot propagation, it is less likely that FXI contributes to hemostasis. , In patients with FXI deficiency, it has been reported that spontaneous bleeding is rarely observed, but the bleeding risk is increased following injuries and surgeries. − More recently, a large retrospective cohort study confirmed that FXI deficiency (FXI activity < 50%) was associated with an increased risk of severe bleeding and clinically relevant nonsevere bleeding, primarily postprocedure . This phenotype is unlike the more frequent spontaneous bleeding found in patients with hemophilia A (factor VIII deficiency) and hemophilia B (factor IX deficiency), , supporting a minor role of FXI in hemostasis.…”

Section: Role Of Factor XI (Fxi) In Thrombosis and Hemostasismentioning

confidence: 99%

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Determination of the Potential Clinical Benefits of Small Molecule Factor XIa Inhibitors in Arterial Thrombosis

Wichaiyo

Parichatikanond

Visansirikul

et al. 2023

ACS Pharmacol. Transl. Sci.

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Anticoagulants are the mainstay for the prevention and treatment of thrombosis. However, bleeding complications remain a primary concern. Recent advances in understanding the contribution of activated factor XI (FXIa) in arterial thrombosis with a limited impact on hemostasis have led to the development of several FXIa-targeting modalities. Injectable agents including monoclonal antibodies and antisense oligonucleotides against FXIa have been primarily studied in venous thrombosis. The orally active small molecules that specifically inhibit the active site of FXIa are currently being investigated for their antithrombotic activity in both arteries and veins. This review focuses on a discussion of the potential clinical benefits of small molecule FXIa inhibitors, mainly asundexian and milvexian, in arterial thrombosis based on their pharmacological profiles and the compelling results of phase 2 clinical studies. The preclinical and epidemiological basis for the impact of FXIa in hemostasis and arterial thrombosis is also addressed. In recent clinical study results, asundexian appears to reduce ischemic events in patients with myocardial infarction and minor-to-moderate stroke, whereas milvexian possibly provides benefits in patients with minor stroke or high-risk transient ischemic attack (TIA). In addition, asundexian and milvexian had a minor impact on hemostasis even in combination with dual-antiplatelet therapy. Other orally active FXIa inhibitors also produce antithrombotic activity in vivo with low bleeding risk. Therefore, FXIa inhibitors might represent a new class of direct-acting oral anticoagulants (DOACs) for the treatment of thrombosis, although the explicit clinical positions of asundexian and milvexian in patients with ischemic stroke, high-risk TIA, and coronary artery disease require confirmation from the outcomes of ongoing phase 3 trials.

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Section: Hemostasismentioning

confidence: 95%

Section: Role Of Factor XI (Fxi) In Thrombosis and Hemostasismentioning

confidence: 99%

Determination of the Potential Clinical Benefits of Small Molecule Factor XIa Inhibitors in Arterial Thrombosis

Wichaiyo

Parichatikanond

Visansirikul

et al. 2023

ACS Pharmacol. Transl. Sci.

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“…Hemophilia patients experience spontaneous and post traumatic bleeds usually in muscles and jointsbut life-threatening intracranial bleeds can also occur which greatly increase the mortality of the hemophiliacs 4 . Over the last few decades, the quality of life of hemophiliacs has improved drastically due to availability of factor replacement therapies but as these therapies are expensive,most patients cannot afford them 5 . The main aim of treatment is to maintain hemostasis and prevent and control active bleed through factor replacement.…”

Section: Introductionmentioning

confidence: 99%

“…Researchers in Pakistan have conducted studies with similar methods, with one study reporting 650 cases in a just 9 months 4 . Most cases of peritonitis are caused by a gastrointestinal perforation or anastomotic leak 5 . In the case of peritonitis, anaerobes and gramnegative organisms are mostly responsible for sepsis and morbidity due to the overactive inflammatory cascade brought on by the release of endotoxins 5 .Clinical evidence is used to identify peritonitis.…”

Section: Introductionmentioning

confidence: 99%

“…Most cases of peritonitis are caused by a gastrointestinal perforation or anastomotic leak 5 . In the case of peritonitis, anaerobes and gramnegative organisms are mostly responsible for sepsis and morbidity due to the overactive inflammatory cascade brought on by the release of endotoxins 5 .Clinical evidence is used to identify peritonitis. Diagnosis can be achieved via upright plain x-ray of the abdomen, USG, or CT scan.…”

Section: Introductionmentioning

confidence: 99%

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Development Of Inhibitors In Hemophilia A Patients Receiving Different Treatment Options

Imitiaz,

Minhas,

Ayesha Younus

et al. 2023

Proceedings S.Z.M.C

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Introduction: Hemophilia A is the most common inherited bleeding disorder in Pakistan. Apart from frequent bleeds and joint deformities, the formation of inhibitors is the major complication of hemophilia. Inhibitors are antibodies formed against the coagulation factor. The purpose of this study is to determine the incidence of inhibitor development in hemophilia A patient receiving different treatment modalities namely fresh frozen plasma (FFP)/cryoprecipitate, plasma derived FVIII concentrates and long-acting recombinant FVIII concentrates. Aims & Objectives: To assess the development of inhibitors in hemophilia A patients receiving treatment modalities. Place and Duration of Study: The study was conducted at Chughtai Institute of Pathology from November 2021 to November 2023. Material &Methods: A total of 75 registered patients of the Hemophilia Patient Welfare Society, Lahore and Sundas Foundation were initially screened for FVIII inhibitors. Those who were found negative for these inhibitors were included in the study and then divided into three equal groups of 25 patients each. One group received only FFP/cryoprecipitate for treatment, the other group received plasma derived FVIII concentrates and the last group received recombinant factor VIII concentrates. These patients were observed for two years i.e after 15-20 exposures and following this period their inhibitors screening was done again to establish the effect of different treatment modalities on inhibitor development. The data collected was entered and analyzed by SPSS-21, a p-value of ?0.05 was considered significant. Results: Our study showed that only 3 patients out of 25, receiving FFP as treatment modality developed inhibitors. Whereas, none of the patients receiving plasma derived FVIII concentrates and recombinant factor VIII concentrates developed inhibitors. The P value calculated is 0.044 which is significant. Conclusion: Plasma derived FVIII concentrates and recombinant FVIII concentrates are safer as compared to FFP/Cryoprecipitate and have lesser risk for inhibitor development.

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Two pediatric cases of severe hemophilia A in which emicizumab prophylaxis failed to prevent traumatic extra-articular hemorrhage

et al. 2022

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Life-threatening bleeding in patients with hemophilia (PWH): a 10-year cohort study in Dakar, Senegal

Cited by 7 publications

References 20 publications

Determination of the Potential Clinical Benefits of Small Molecule Factor XIa Inhibitors in Arterial Thrombosis

Determination of the Potential Clinical Benefits of Small Molecule Factor XIa Inhibitors in Arterial Thrombosis

Development Of Inhibitors In Hemophilia A Patients Receiving Different Treatment Options

Two pediatric cases of severe hemophilia A in which emicizumab prophylaxis failed to prevent traumatic extra-articular hemorrhage

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