Coagulation factor XIII - Pathophysiology, clinic and therapy of factor XIII deficiency;Der Gerinnungsfaktor XIII - Pathophysiologie, Klinik und Therapie von Mangelzuständen

Anasthesiologie Intensivmedizin Notfallmedizin Schmerztherapie

Volumn 50, Issue 11-12, 2015, Pages 684-690

  Weber, Christian Friedrich ^a   Adam, Elisabeth Hannah ^a   Pape, Andreas ^a   Jöst, Marina ^b   Meybohm, Patrick ^a   Schmitz, Katja ^b   Zacharowski, Kai ^a   Hermann, Martin ^c   Fries, Dietmar ^c  

^a UNIVERSITY HOSPITAL FRANKFURT   (Germany)

^b DARMSTADT UNIVERSITY OF TECHNOLOGY   (Germany)

^c INNSBRUCK MEDICAL UNIVERSITY   (Austria)

Author keywords

coagulation; coagulopathy; factor XIII; factor XIII deficiency; hemostasis

Indexed keywords

BLOOD CLOTTING FACTOR 13; IMMUNOSUPPRESSIVE AGENT;

ARTICLE; BLOOD CLOTTING FACTOR 13 DEFICIENCY; BLOOD CLOTTING TEST; BLOOD COAGULATION DISORDERS; BLOOD TRANSFUSION; COMPLICATION; FACTOR XIII DEFICIENCY; GENETIC SCREENING; HEMORRHAGE; HUMAN; PROCEDURES;

BLOOD COAGULATION DISORDERS; BLOOD COAGULATION TESTS; BLOOD TRANSFUSION; FACTOR XIII DEFICIENCY; GENETIC TESTING; HEMORRHAGE; HUMANS; IMMUNOSUPPRESSIVE AGENTS;

EID: 84949505674     PISSN: 09392661     EISSN: 14391074     Source Type: Journal    
DOI: 10.1055/s-0041-105951     Document Type: Article

References (90)

1. Monroe D. M., Hoffman M. What does it take to make the perfect clot? ArteriosclerThrombVascBiol: 2006; 26 41 48
2. Komaromi I., Bagoly Z., Muszbek L. Factor XIII: novel structural and functional aspects. J ThrombHaemost: 2011; 9 9 20
3. Schwartz M. L., Pizzo S. V., Hill R. L., McKee P. A. Human Factor XIII from plasma and platelets. Molecular weights, subunit structures, proteolytic activation, and cross-linking of fibrinogen and fibrin. J BiolChem: 1973; 248 1395 407
4. Muszbek L., Adany R., Mikkola H. Novel aspects of blood coagulation factor XIII. I. Structure, distribution, activation, and function. Crit Rev Clin Lab Sci: 1996; 33 357 421
5. Schwartz M. L., Pizzo S. V., Hill R. L., McKee P. A. The subunit structures of human plasma and platelet factor XIII (fibrin-stabilizing factor). J BiolChem: 1971; 246 5851 5854
6. Katona E., Penzes K., Csapo A. et al. Interaction of factor XIII subunits. Blood: 2014; 123 1757 1763
7. Mary A., Achyuthan K. E., Greenberg C. S. b-chains prevent the proteolytic inactivation of the a-chains of plasma factor XIII. BiochimBiophysActa: 1988; 966 328 335
8. Souri M., Osaki T., Ichinose A. The non-catalytic B subunit of coagulation factor XIII accelerates fibrin cross-linking. J BiolChem: 2015; 290 12027 12039
9. Muszbek L., Bereczky Z., Bagoly Z. et al. Factor XIII: a coagulation factor with multiple plasmatic and cellular functions. Physiol Rev: 2011; 91 931 972
10. Dufner G. S., Marbet G. A. Factor XIII in man: a review. Hamostaseologie: 2002; 22 11 19
11. Adany R. Intracellular factor XIII: cellular distribution of factor XIII subunit a in humans. SeminThrombHemost: 1996; 22 399 408
12. Kradin R. L., Lynch G. W., Kurnick J. T. et al. Factor XIII A is synthesized and expressed on the surface of U937 cells and alveolar macrophages. Blood: 1987; 69 778 785
13. Muszbek L., Yee V. C., Hevessy Z. Blood coagulation factor XIII: structure and function. Thromb Res: 1999; 94 271 305
14. Board P. G., Losowsky M. S., Miloszewski K. J. Factor XIII: inherited and acquired deficiency. Blood Rev: 1993; 7 229 242
15. Schroeder V., Kohler H. P. New developments in the area of factor XIII. J ThrombHaemost: 2013; 11 234 244
16. Schroeder V., Vuissoz J. M., Caflisch A., Kohler H. P. Factor XIII activation peptide is released into plasma upon cleavage by thrombin and shows a different structure compared to its bound form. ThrombHaemost: 2007; 97 890 898
17. Lord S. T. Fibrinogen and fibrin: scaffold proteins in hemostasis. CurrOpinHematol: 2007; 14 236 241
18. McKee P. A., Mattock P., Hill R. L. Subunit structure of human fibrinogen, soluble fibrin, and cross-linked insoluble fibrin. Proc Natl AcadSci U S A: 1970; 66 738 744
19. Sakata Y., Aoki N. Significance of cross-linking of alpha 2-plasmin inhibitor to fibrin in inhibition of fibrinolysis and in hemostasis. J Clin Invest: 1982; 69 536 542
20. Tamaki T., Aoki N. Cross-linking of alpha 2-plasmin inhibitor to fibrin catalyzed by activated fibrin-stabilizing factor. J BiolChem: 1982; 257 14767 14772
21. Fraser S. R., Booth N. A., Mutch N. J. The antifibrinolytic function of factor XIII is exclusively expressed through alpha(2)-antiplasmin cross-linking. Blood: 2011; 117 6371 6374
22. Dirkmann D., Gorlinger K., Gisbertz C. et al. Factor XIII and tranexamic acid but not recombinant factor VIIa attenuate tissue plasminogen activator-induced hyperfibrinolysis in human whole blood. AnesthAnalg: 2012; 114 1182 1188
23. Shenkman B., Einav Y., Livnat T. et al. In vitro evaluation of clot quality and stability in a model of severe thrombocytopenia: effect of fibrinogen, factor XIII and thrombin-activatable fibrinolysis inhibitor. Blood Transfus: 2014; 12 78 84
24. Noll T., Wozniak G. Factor XIII and endothelial barrier function. Hamostaseologie: 2002; 22 28 31
25. Noll T., Wozniak G., McCarson K. et al. Effect of factor XIII on endothelial barrier function. J Exp Med: 1999; 189 1373 1382
26. Naderi M., Dorgalaleh A., Alizadeh S. et al. Clinical manifestations and management of life-threatening bleeding in the largest group of patients with severe factor XIII deficiency. Int J Hematol: 2014; 100 443 449
27. Hoetzenecker W., Guenova E., Mitev V. et al. Treatment of pyoderma gangrenosum with topical factor XIII. Eur J Dermatol: 2013; 23 653 657
28. Andersson C., Kvist P. H., McElhinney K. et al. Factor XIII transglutaminase supports the resolution of mucosal damage in experimental colitis. PLoS One: 2015; 10
29. Bregenzer N., Caesar I., Andus T. et al. Lack of clinical efficacy of additional factor XIII treatment in patients with steroid refractory colitis. The Factor XIII Study Group. Z Gastroenterol: 1999; 37 999 1004
30. Floridon C., Nielsen O., Holund B. et al. Does plasminogen activator inhibitor-1 (PAI-1) control trophoblast invasion? A study of fetal and maternal tissue in intrauterine, tubal and molar pregnancies. Placenta: 2000; 21 754 762
31. Mello G., Parretti E., Gensini F. et al. Maternal-fetal flow, negative events, and preeclampsia: role of ACE I/D polymorphism. Hypertension: 2003; 41 932 937
32. Koseki-Kuno S., Yamakawa M., Dickneite G., Ichinose A. Factor XIII A subunit-deficient mice developed severe uterine bleeding events and subsequent spontaneous miscarriages. Blood: 2003; 102 4410 4412
33. Dossenbach-Glaninger A., van Trotsenburg M., Dossenbach M. et al. Plasminogen activator inhibitor 1 4G/5G polymorphism and coagulation factor XIII Val34Leu polymorphism: impaired fibrinolysis and early pregnancy loss. ClinChem: 2003; 49 1081 1086
34. Asahina T., Kobayashi T., Okada Y. et al. Maternal blood coagulation factor XIII is associated with the development of cytotrophoblastic shell. Placenta: 2000; 21 388 393
35. Boda Z., Pfliegler G., Muszbek L. et al. Congenital factor XIII deficiency with multiple benign breast tumours and successful pregnancy with substitutive therapy. A casereport. Haemostasis: 1989; 19 348 352
36. Kobayashi T., Terao T., Kojima T. et al. Congenital factor XIII deficiency with treatment of factor XIII concentrate and normal vaginal delivery. GynecolObstet Invest: 1990; 29 235 238
37. Bremme K. A. Haemostatic changes in pregnancy. Best Pract Res ClinHaematol: 2003; 16 153 168
38. Karlsson O., Jeppsson A., Hellgren M. A longitudinal study of factor XIII activity, fibrinogen concentration, platelet count and clot strength during normal pregnancy. Thromb Res: 2014; 134 750 752
39. Sharief L. T., Lawrie A. S., Mackie I. J. et al. Changes in factor XIII level during pregnancy. Haemophilia: 2014; 20 144 148
40. Wang Z., Wilhelmsson C., Hyrsl P. et al. Pathogen entrapment by transglutaminase - a conserved early innate immune mechanism. PLoSPathog: 2010; 6
41. Loof T. G., Morgelin M., Johansson L. et al. Coagulation, an ancestral serine protease cascade, exerts a novel function in early immune defense. Blood: 2011; 118 2589 2598
42. Biswas A., Ivaskevicius V., Seitz R. et al. An update of the mutation profile of Factor 13 A and B genes. Blood Rev: 2011; 25 193 204
43. Kohler H. P., Ariens R. A., Whitaker P., Grant P. J. A common coding polymorphism in the FXIII A-subunit gene (FXIIIVal34Leu) affects cross-linking activity. ThrombHaemost: 1998; 80 704 704
44. Ariens R. A., Philippou H., Nagaswami C. et al. The factor XIII V34L polymorphism accelerates thrombin activation of factor XIII and affects cross-linked fibrin structure. Blood: 2000; 96 988 995
45. Trumbo T. A., Maurer M. C. Examining thrombin hydrolysis of the factor XIII activation peptide segment leads to a proposal for explaining the cardioprotective effects observed with the factor XIII V34L mutation. J BiolChem: 2000; 275 20627 20631
46. Antalfi B., Pongracz E., Csiki Z. et al. Factor XIII-A subunit Val34Leu polymorphism in fatal hemorrhagic stroke. Int J Lab Hematol: 2013; 35 88 91
47. Voko Z., Bereczky Z., Katona E. et al. Factor XIII Val34Leu variant protects against coronary artery disease. A meta-analysis. ThrombHaemost: 2007; 97 458 463
48. Kreutz R. P., Bitar A., Owens J. et al. Factor XIII Val34Leu polymorphism and recurrent myocardial infarction in patients with coronary artery disease. J Thromb Thrombolysis: 2014; 38 380 387
49. Kohler H. P., Schroder V. Role of coagulation factor XIII in cardio- and cerebrovascular diseases. Hamostaseologie: 2002; 22 53 58
50. Salazar-Sanchez L., Leon M. P., Cartin M. et al. The FXIIIVal34Leu, common and risk factors of venous thrombosis in early middle-age Costa Rican patients. Cell BiochemFunct: 2007; 25 739 745
51. Endler G., Funk M., Haering D. et al. Is the factor XIII 34Val/Leu polymorphism a protective factor for cerebrovascular disease? Br J Haematol: 2003; 120 310 314
52. Shemirani A. H., Antalfi B., Pongracz E. et al. Factor XIII-A subunit Val34Leu polymorphism in fatal atherothrombotic ischemic stroke. Blood Coagul Fibrinolysis: 2014; 25 364 368
53. Weber C. F., Sanders J. O., Friedrich K. et al. Role of thrombelastometry for the monitoring of factor XIII. A prospective observational study in neurosurgical patients. Hamostaseologie: 2011; 31 111 117
54. Katona E., Penzes K., Molnar E., Muszbek L. Measurement of factor XIII activity in plasma. ClinChem Lab Med: 2012; 50 1191 1202
55. Oertel K., Hunfeld A., Specker E. et al. A highly sensitive fluorometric assay for determination of human coagulation factor XIII in plasma. Anal Biochem: 2007; 367 152 158
56. Naderi M., Dorgalaleh A., Tabibian S. et al. Current understanding in diagnosis and management of factor XIII deficiency. Iran J Ped HematolOncol: 2013; 3 164 172
57. Fickenscher K., Aab A., Stuber W. A photometric assay for blood coagulation factor XIII. ThrombHaemost: 1991; 65 535 540
58. Anwar R., Gallivan L., Edmonds S. D., Markham A. F. Genotype/phenotype correlations for coagulation factor XIII: specific normal polymorphisms are associated with high or low factor XIII specific activity. Blood: 1999; 93 897 905
59. Duckert F., Jung E., Shmerling D. H. A hitherto undescribed congenital haemorrhagic diathesis probably due to fibrin stabilizing factor deficiency. ThrombDiathHaemorrh: 1960; 5 179 186
60. Odame J. E., Chan A. K., Wu J. K., Breakey V. R. Factor XIII deficiency management: a review of the literature. Blood Coagul Fibrinolysis: 2014; 25 199 205
61. Anwar R., Miloszewski K. J. Factor XIII deficiency. Br J Haematol: 1999; 107 468 484
62. Ichinose A. Physiopathology and regulation of factor XIII. ThrombHaemost: 2001; 86 57 65
63. Peyvandi F., Palla R., Menegatti M. et al. Coagulation factor activity and clinical bleeding severity in rare bleeding disorders: results from the European Network of Rare Bleeding Disorders. J ThrombHaemost: 2012; 10 615 621
64. Biswas A., Ivaskevicius V., Thomas A., Oldenburg J. Coagulation factor XIII deficiency. Diagnosis, prevalence and management of inherited and acquired forms. Hamostaseologie: 2014; 34 160 166
65. Nussbaum M., Morse B. S. Plasma fibrin stabilizing factor activity in various diseases. Blood: 1964; 23 669 678
66. Lorand L., Velasco P. T., Murthy S. N. et al. Autoimmune antibody in a hemorrhagic patient interacts with thrombin-activated factor XIII in a unique manner. Blood: 1999; 93 909 917
67. Lorand L., Velasco P. T., Hill J. M. et al. Intracranial hemorrhage in systemic lupus erythematosus associated with an autoantibody against actor XIII. ThrombHaemost: 2002; 88 919 923
68. Souri M., Osaki T., Ichinose A. Anti-factor XIII A subunit (FXIII-A) autoantibodies block FXIII-A2 B2 assembly and steal FXIII-A from native FXIII-A2 B2. J ThrombHaemost: 2015; 13 802 814
69. Ichinose A., Osaki T., Souri M. Clinical features of 32 new Japanese cases with autoimmune haemorrha-philia due to anti-factor XIII antibodies. Haemophilia: 2015; 21 653 658
70. Egbring R., Kroniger A., Seitz R. Factor XIII deficiency: pathogenic mechanisms and clinical significance. SeminThrombHemost: 1996; 22 419 425
71. Haas T., Korte W., Spielmann N. et al. Perioperative course of FXIII in children undergoing major surgery. PaediatrAnaesth: 2012; 22 641 646
72. Gerlach R., Tolle F., Raabe A. et al. Increased risk for postoperative hemorrhage after intracranial surgery in patients with decreased factor XIII activity: implications of a prospective study. Stroke: 2002; 33 1618 1623
73. Bockeria L. A., Samsonova N. N., Yurlov I. A. et al. Dynamics of factor XIII levels after open heart surgery for congenital heart defects: do cyanotic and acyanotic patients differ? PediatrCardiol: 2014; 35 1108 1115
74. Kalbhenn J., Wittau N., Schmutz A. et al. Identification of acquired coagulation disorders and effects of target-controlled coagulation factor substitution on the incidence and severity of spontaneous intracranial bleeding during veno-venous ECMO therapy. Perfusion: 2015; 30 675 682
75. Theusinger O. M., Baulig W., Seifert B. et al. Changes in coagulation in standard laboratory tests and ROTEM in trauma patients between on-scene and arrival in the emergency department. AnesthAnalg: 2015; 120 627 635
76. Seitz R., Duckert F., Lopaciuk S. et al. ETRO Working Party on Factor XIII questionnaire on congenital factor XIII deficiency in Europe: status and perspectives. Study Group. SeminThrombHemost: 1996; 22 415 418
77. Ivaskevicius V., Biswas A., Bevans C. et al. Identification of eight novel coagulation factor XIII subunit A mutations: implied consequences for structure and function. Haematologica: 2010; 95 956 962
78. Acharya S. S., Coughlin A., Dimichele D. M. Rare Bleeding Disorder Registry: deficiencies of factors II, V, VII, X, XIII, fibrinogen and dysfibrinogenemias. J ThrombHaemost: 2004; 2 248 256
79. Biswas A., Ivaskevicius V., Thomas A. et al. Eight novel F13A1 gene missense mutations in patients with mild FXIII deficiency: in silico analysis suggests changes in FXIII-A subunit structure/function. Ann Hematol: 2014; 93 1665 1676
80. Wettstein P., Haeberli A., Stutz M. et al. Decreased factor XIII availability for thrombin and early loss of clot firmness in patients with unexplained intraoperative bleeding. AnesthAnalg: 2004; 99 1564 1569
81. Korte W. C., Szadkowski C., Gahler A. et al. Factor XIII substitution in surgical cancer patients at high risk for intraoperative bleeding. Anesthesiology: 2009; 110 239 245
82. Godje O., Gallmeier U., Schelian M. et al. Coagulation factor XIII reduces postoperative bleeding after coronary surgery with extracorporeal circulation. ThoracCardiovascSurg: 2006; 54 26 33
83. Fadoo Z., Merchant Q., Rehman K. A. New developments in the management of congenital Factor XIII deficiency. J Blood Med: 2013; 4 65 73
84. Kerlin B., Brand B., Inbal A. et al. Pharmacokinetics of recombinant factor XIII at steady state in patients with congenital factor XIII A-subunit deficiency. J ThrombHaemost: 2014; 12 2038 2043
85. Ashley C., Chang E., Davis J. et al. Efficacy and safety of prophylactic treatment with plasma-derived factor XIII concentrate (human) in patients with congenital factor XIII deficiency. Haemophilia: 2015; 21 102 108
86. Korte W. Fibrin monomer and factor XIII: a new concept for unexplained intraoperative coagulopathy. Hamostaseologie: 2006; 26 30 35
87. Weber C. F., Zacharowski K., Meybohm P. et al. Hemotherapy algorithms for coagulopathic cardiac surgery patients. Clin Lab: 2014; 60 1059 1063
88. Weber C. F., Zacharowski K., Brun K. et al. Basisalgorithmus für "Point-of-Care basierte Hämotherapie: Perioperative Versorgung koagulopathischer Patienten. Anaesthesist: 2013; 62 464 472
89. Kozek-Langenecker S. A., Afshari A., Albaladejo P. et al. Management of severe perioperative bleeding: guidelines from the European Society of Anaesthesiology. Eur J Anaesthesiol: 2013; 30 270 382
90. Scharrer I. Replacement therapy of coagulation factor preparations: antithrombin, FFP, PPSB, FXIII, rFVIIa. Hamostaseologie: 2005; 25 209 212