Spontaneous factor VIII inhibitors may be responsible for severe life-threatening haemorrhages. It is reported on two patients with inhibitors directed against factor VIII: C. In one female patient a rheumatoid arthritis could be made evident as basic disease. Diagnostics, therapy and course are reported and discussed.
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J Pharmacol Exp Ther
February 2025
Versiti Blood Research Institute, Milwaukee, Wisconsin. Electronic address:
Most factor VIII (FVIII) in circulation exists in a complex with von Willebrand factor (vWF). The interaction between FVIII and vWF is vital for normal hemostatic function, and disruptions in this interaction can lead to bleeding disorders such as von Willebrand disease or hemophilia. However, the impact of pathological mutations on the binding between FVIII and vWF remains largely uncharacterized.
View Article and Find Full Text PDFArch Rheumatol
December 2024
Division of Pediatric Hematology, Lösante Hospital, Ankara, Türkiye.
Objectives: This study aimed to determine whether there are commonly occurring hematologic causes that may lead to thrombosis in patients recently diagnosed with immunoglobulin A vasculitis (IgAV).
Patients And Methods: The prospective study enrolled 107 pediatric patients diagnosed with IgAV and 98 healthy age- and sex-matched controls. Patients with IgAV who were treated at a single center between February 2016 and June 2022 were evaluated for the prevalence and clinical relevance of thrombophilic gene mutations and other prothrombotic risk factors, as well as coagulation test indices.
J Thromb Haemost
March 2025
Unite d'Hemostase Clinique, Centre de Reference de l'Hemophilie, Hopital Louis Pradel, Lyon, France; Universite Claude Bernard Lyon 1, UR4609 Hemostase & Thrombose, Lyon, France. Electronic address:
Background: For every man diagnosed with hemophilia, approximately 1.6 women are expected to be carriers. Carriers are classified based on their Factor VIII (FVIII) levels and symptoms, ranging from asymptomatic to mild, moderate or severe symptoms.
View Article and Find Full Text PDFJ Thromb Haemost
March 2025
Herman B Wells Center for Pediatric Research, Indiana University School of Medicine, Indianapolis, IN 46202. Electronic address:
Background: The development of inhibitory antibodies (inhibitors) is a serious complication in the treatment of hemophilia A with clotting factor VIII (FVIII) replacement therapy. Inhibitor formation critically depends on T cell help and modulation by regulatory T cells (Tregs).
Objective: In this study, we evaluated the F5111 immunocytokine (IC), a single chain fusion between the human interleukin-2 (IL-2) cytokine and an IL-2 antibody that biases cytokine activity towards cells with high IL-2 receptor alpha (IL-2Rα) expression, leading to extended IL-2 half-life and selective expansion of Tregs.
Thromb Haemost
March 2025
The Province and Ministry Co-sponsored Collaborative Innovation Center for Medical Epigenetics, Department of Physiology and Pathophysiology, Tianjin Medical University, Tianjin, China.
Patients with coronavirus disease 2019 (COVID-19) are at high risk of developing a hypercoagulable state and thrombosis. The von Willebrand factor (vWF) produced by endothelial cells (ECs) is a critical thrombosis regulator. We previously found that cytoskeleton-associated protein 4 (CKAP4) is a novel receptor for the spike protein of severe acute respiratory syndrome coronavirus-2 and is involved in COVID-19-associated coagulopathy.
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