Coagulation Factor XI (FXI) and Factor XII (FXII) deficiencies are rare. FXI deficiency is associated with a bleeding disorder, while FXII deficiency is not, but both can cause chronic prolongation of activated partial thromboplastin time and impair thrombus formation, posing great challenges for hemodialysis anticoagulation. Traditionally, heparin or low-molecular-weight heparins (LMWHs) are not considered a safe anticoagulation option for patients with increased bleeding risk. In this context, FXI and FXII have received substantial attention as targets for new anticoagulants. We present the case of a 68-year-old woman with combined FXI and FXII deficiencies who successfully underwent hemodialysis with anticoagulation using a low dose of LMWHs. This case highlights that FXI and FXII deficiencies are associated with anticoagulant effects, which can reduce the dosage of anticoagulant during hemodialysis. With careful monitoring, an appropriate dosage of LMWHs is still an acceptable option for patients with a bleeding risk.
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http://dx.doi.org/10.1111/sdi.13219 | DOI Listing |
J Pediatr Hematol Oncol
December 2024
Pediatrics Clinic, Bakirkoy Dr. Sadi Konuk Training and Research Hospital, University of Health Sciences, Istanbul, Turkey.
Background: Rare factor deficiency (RFD) is characterized by a deficiency of factor (F)I, FII, FV, FVII, FX, FXI, FXII, FXIII, or a combined deficiency of FV+FVIII or vitamin K-dependent factors. The prevalence of RFD ranges from 1/1,000,000 to 3,000,000. Combined deficiencies of vitamin K-related factors have been described in 30 families worldwide, and these patients can present with a wide range of clinical symptoms, from mucocutaneous bleeding to life-threatening symptoms such as central nervous system and gastrointestinal bleeding.
View Article and Find Full Text PDFHaemophilia
November 2024
Faculty of Medicine, Geneva, Switzerland.
Introduction: Rare coagulation disorders (RCDs) constitute an important health risk. Data on epidemiology, quality of life (QoL), access to care, and impact of the ongoing economic crisis on RCDs in Lebanon is limited.
Aim: We aimed to address these gaps by evaluating effect of the crisis on the management of RCDs.
PLoS Comput Biol
November 2024
CSL Ltd, Bio21 Institute, Melbourne, Victoria, Australia.
Background: The kallikrein-kinin system (KKS) is a complex biochemical pathway that plays a crucial role in regulating several physiological processes, including inflammation, coagulation, and blood pressure. Dysregulation of the KKS has been associated with several pathological conditions such as hereditary angioedema (HAE), hypertension, and stroke. Developing an accurate quantitative model of the KKS may provide a better understanding of its role in health and disease and facilitate the rapid and targeted development of effective therapies for KKS-related disorders.
View Article and Find Full Text PDFBiomolecules
October 2024
Department of Dermatology, Faculty of Medicine, University of Medicine and Pharmacy of Craiova, 200349 Craiova, Romania.
Int J Mol Sci
October 2024
Department of Interdisciplinary Oncology, Louisiana State University Health Sciences Center (LSUHSC), New Orleans, LA 70112, USA.
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