RT Journal Article
T1 The vascular endothelium and coagulation: homeostasis, disease, and treatment, with a focus on the von willebrand factor and factors VIII and V
A1 Serrano, Luis Javier
A1 Sánchez, María José
A1 De Pablo Moreno, Juan Andrés
A1 Revuelta Rueda, Luis
A1 Liras Martín, Antonio
AB The vascular endothelium has several important functions, including hemostasis.  The homeostasis of hemostasis is based on a fine balance between procoagulant and anticoagulant pro-teins and between fibrinolytic and antifibrinolytic ones. Coagulopathies are characterized by a mutation-induced alteration of the function of certain coagulation factors or by a disturbed balance between the mechanisms responsible for regulating coagulation. Homeostatic therapies consist in replacement and nonreplacement treatments or in the administration of antifibrinolytic agents. Rebalancing products reestablish hemostasis by inhibiting natural anticoagulant pathways. These agents include monoclonal antibodies, such as concizumab and marstacimab, which target the tissue factor pathway inhibitor; interfering RNA therapies, such as fitusiran, which targets antithrombin III; and protease inhibitors, such as serpinPC, which targets active protein C. In cases of throm- bophilia (deficiency of protein C, protein S, or factor V Leiden), treatment may consist in direct oral anticoagulants, replacement therapy (plasma or recombinant ADAMTS13) in cases of a congenital deficiency of ADAMTS13, or immunomodulators (prednisone) if the thrombophilia is autoimmune. Monoclonal-antibody-based anti-vWF immunotherapy (caplacizumab) is used in the context of severe thrombophilia, regardless of the cause of the disorder. In cases of disseminated intravascular coagulation, the treatment of choice consists in administration of antifibrinolytics, all-trans-retinoic acid, and recombinant soluble human thrombomodulin.
PB MDPI
SN 1422-0067
YR 2022
FD 2022-07-27
LK https://hdl.handle.net/20.500.14352/95126
UL https://hdl.handle.net/20.500.14352/95126
LA eng
NO Author ContributionsConceptualization, A.L. and J.A.D.P.-M.; figure preparation, A.L. and J.A.D.P.-M.; writing—original draft preparation, A.L., J.A.D.P.-M., L.J.S., and M.J.S.; writing—review and editing, A.L., J.A.D.P.-M., L.J.S., M.J.S., and L.R. A.L. is the principal investigator and was responsible for obtaining the funds required. All authors have read and agreed to the published version of the manuscript.
NO Association for Research and Cure of Factor V deficiency (ASDEFAV/2021-23)
NO Universidad Complutense de Madrid/Banco Santander (CT63/19-CT64/19)
NO Junta de Andalucía (PAI-BIO/295)
DS Docta Complutense
RD 9 abr 2025