Abnormal plasma clot structure and stability distinguish bleeding risk in patients with severe factor XI deficiency
作者： M. Zucker , U. Seligsohn , O. Salomon , A. S. Wolberg
DOI: 10.1111/JTH.12600
关键词: Gastroenterology 、 Factor XIII 、 Von Willebrand factor 、 Fibrinolysis 、 Asymptomatic 、 Factor XI 、 Internal medicine 、 In patient 、 Medicine 、 Severe bleeding 、 Surgery 、 Plasma clot
摘要: Background Factor XI (FXI) deficiency is a rare autosomal recessive disorder. Many patients with even very low FXI levels (<20 IU/dL) are asymptomatic or exhibit only mild bleeding, whereas others experience severe bleeding usually following trauma. Neither antigen nor activity predicts risk in FXI-deficient patients.
sci-hub.se 参考文章(35)
Kevin M. Cawthern, Cornelis van ‘t Veer, Jennifer B. Lock, Maria E. DiLorenzo, Richard F. Branda, Kenneth G. Mann, Blood Coagulation in Hemophilia A and Hemophilia C Blood. ,vol. 91, pp. 4581- 4592 ,(1998) , 10.1182/BLOOD.V91.12.4581
PA von dem Borne, JC Meijers, BN Bouma, Feedback activation of factor XI by thrombin in plasma results in additional formation of thrombin that protects fibrin clots from fibrinolysis Blood. ,vol. 86, pp. 3035- 3042 ,(1995) , 10.1182/BLOOD.V86.8.3035.3035
Paul Guéguen, Hubert Galinat, Marie-Thérèse Blouch, Françoise Bridey, Jérôme Duchemin, Grégoire Le Gal, Jean-François Abgrall, Brigitte Pan-Petesch, Biological determinants of bleeding in patients with heterozygous factor XI deficiency. British Journal of Haematology. ,vol. 156, pp. 245- 251 ,(2012) , 10.1111/J.1365-2141.2011.08945.X
F. PEYVANDI, D. DI MICHELE, P. H. B. BOLTON-MAGGS, C. A. LEE, A. TRIPODI, A. SRIVASTAVA, , Classification of rare bleeding disorders (RBDs) based on the association between coagulant factor activity and clinical bleeding severity Journal of Thrombosis and Haemostasis. ,vol. 10, pp. 1938- 1943 ,(2012) , 10.1111/J.1538-7836.2012.04844.X
MV Ragni, D Sinha, F Seaman, JH Lewis, JA Spero, PN Walsh, Comparison of bleeding tendency, factor XI coagulant activity, and factor XI antigen in 25 factor XI-deficient kindreds Blood. ,vol. 65, pp. 719- 724 ,(1985) , 10.1182/BLOOD.V65.3.719.719
L. RUGERI, F. QUÉLIN, B. CHATARD, P. DE MAZANCOURT, C. NEGRIER, Y. DARGAUD, Thrombin generation in patients with factor XI deficiency and clinical bleeding risk. Haemophilia. ,vol. 16, pp. 771- 777 ,(2010) , 10.1111/J.1365-2516.2010.02246.X
A. S. Wolberg, D. A. Gabriel, M. Hoffman, Analyzing fibrin clot structure using a microplate reader. Blood Coagulation & Fibrinolysis. ,vol. 13, pp. 533- 539 ,(2002) , 10.1097/00001721-200209000-00008
B. Blombäck, K. Carlsson, B. Hessel, A. Liljeborg, R. Procyk, N. Åslund, Native fibrin gel networks observed by 3D microscopy, permeation and turbidity Biochimica et Biophysica Acta (BBA) - Protein Structure and Molecular Enzymology. ,vol. 997, pp. 96- 110 ,(1989) , 10.1016/0167-4838(89)90140-4
S. Berliner, I. Horowitz, U. Martinowitz, B. Brenner, U. Seligsohn, Dental surgery in patients with severe factor XI deficiency without plasma replacement. Blood Coagulation & Fibrinolysis. ,vol. 3, pp. 465- 468 ,(1992) , 10.1097/00001721-199203040-00014
Laszlo Bajzar, Reg Manuel, Michael E. Nesheim, Purification and Characterization of TAFI, a Thrombin-activable Fibrinolysis Inhibitor Journal of Biological Chemistry. ,vol. 270, pp. 14477- 14484 ,(1995) , 10.1074/JBC.270.24.14477