What happens to coagulation thrombosis mechanism in liver diseases?

Abstract

Children with liver disease present complex and varied acquired coagulation abnormalities Liver is the place where clotting factors are produced as well as inhibitors of coagulation and fibrinolytic system components Although 75 85 of patients with liver disease show abnormal laboratory values only 10 15 will have clinical bleeding symptoms as liver has a large factor reserve capacity This paper will review acute and chronic liver disease and the hemostatic alterations Treatment of the coagulopaty of liver disease is usually replacement of lacking factor and supportive care Liver transplantation which is very common recently and its affects on hematological balance will also summarized Turk Arch Ped 2013; 48: 94 101

Keywords

• Hemorrage, liver disease, thrombosis, treatment

Karaciğer hastalıklarında pıhtılaşma tromboz mekanizmasına neler oluyor?

Öz

Karaciğer hastalığı olan çocuklar karışık ve değişken pıhtılaşma bozuklukları gösterirler Pıhtılaşma faktörleri gibi pıhtılaşmayı engelleyen faktörler ve fibrinoliz karaciğerde yapılır Karaciğer hastalarının 75 85 rsquo;inde laboratuvarda bozukluk olsa da bu hastaların 10 15 rsquo;inde klinik kanama bulgusu vardır çünkü bu faktörler için karaciğerin geniş bir birikimi söz konusudur Bu yazıda akut ve kronik karaciğer hastalıklarında karaciğerin hemostatik özelliklerinden bahsedilecektir Karaciğer hastasının kanamasında genellikle eksik olan faktörün yerine konulması ve destek tedavisi önem kazanır Son yıllarda artan sayıda yapılan karaciğer nakli ve hematolojik sonuçlarından da bahsedilecektir.

Anahtar Kelimeler

• Karaciğer hastalıkları
• kanama
• tromboz
• tedavi

References

1. Joist JH, George JN. Hemostatic abnormalities in liver and renal disease. In: Colman RW, Hirsh J, Marder VJ, Clowes AE, George JN, (eds). Hemostasis and thrombosis. Philadelphia, Pa: Lippincott Williams & Wilkins; 2001: 955-73.
2. Monroe DM, Hoffman M. The coagulation cascade in cirrhosis. Clin Liver Dis 2009; 13(1): 1-9.
3. Lisman T, Caldwell SH, Burroughs AK, et al. Hemostasis and thrombosis in patients with liver disease: the ups and downs. J Hepatol 2010 ; 53(2): 362-71.
4. Gugig R, Rosenthal P. Current options for treatment of end-stage liver disease in children. Curr Pediatr Rev 2005; 1: 261-4.
5. Leebeek FW, Kluft C, Knot EA, de Maat MP, Wilson JH. A shift in balance between profibrinolytic and antifibrinolytic factors causes enhanced fibrinolysis in cirrhosis. Gastroenterology 1991; 101: 1382Ferro D, Quintarelli C, Saliola M, et al. Prevalence of hyperfibrinolysis in patients with liver cirrhosis. Fibrinolysis 1993; 7(1): 59-62.
6. Francis RB Jr, Feinstein DI. Clinical significance of accelerated fibrinolysis in liver disease. Haemostasis 1984; 14(6): 460-5.
7. Ben-Ari Z, Osman E, Hutton RA, Burroughs AK. Disseminated intravascular coagulation in liver cirrhosis: fact or fiction? Am J Gastroenterol 1999; 94(10): 2977-82.
8. Ryan BM, Stockbrugger RW, Ryan JM. A pathophysiologic, gastroenterologic, and radiologic approach to the management of gastric varices. Gastroenterology 2004; 126(4): 1175-89.

9. Smalberg JH, Leebeek FW. Superimposed coagulopathic conditions in cirrhosis: infection and endogenous heparinoids, renal failure, and endothelial dysfunction. Clin Liver Dis 2009; 13(1): 33-42.
10. Lisman T, Porte RJ, Leebeek FW, Caldwell SH. Methodological issues with coagulation testing in patients with liver disease. 2006; 4(9): 2061-2.
11. Mannucci PM. Abnormal hemostasis tests and bleeding in chronic liver disease: are they related? No. J Thromb Haemost 2006; 4(4): 721-3.
12. Reverter JC. Abnormal hemostasis tests and bleeding in chronic liver disease: are they related? Yes. J Thromb Haemost 2006; 4(4): 717-20.
13. Lisman T, Caldwell SH, Porte RJ, Leebeek FW. Consequences of abnormal hemostasis tests for clinical practice. J Thromb Haemost 2006 ; 4(9): 2062-3.
14. Cattaneo M, Tenconi PM, Alberca I, Garcia VV, Mannucci PM. Subcutaneous desmopressin (DDAVP) shortens the prolonged bleeding time in patients with liver cirrhosis. Thromb Haemost 1990; 64(3) : 358-60.
15. Friedman EW, Sussman II. Safety of invasive procedures in patients with the coagulopathy of liver disease. Clin Lab Haematol 1989; 11(3): 199-204.
16. Bell BA. Bleeding associated with hepatocelluler disease. Int J Ped Hem/Onc 1994; 1: 53-61.
17. Ferro D, Quintarelli C, Lattuada A, et al. High plasma levels of von Willebrand factor as a marker of endothelial perturbation in cirrhosis: relationship to endotoxemia. Hepatology 1996; 23(6): 1377Nonami T, Yokoyama I, Iwatsuki S, Starzl TE. The incidence of portal vein thrombosis at liver transplantation. Hepatology 1992; 16(5): 1195-8.
18. Northup PG. Hypercoagulation in liver disease. Clin Liver Dis 2009; 13(1): 109-16.
19. Hugenholtz GG, Porte RJ, Lisman T. The platelet and platelet function testing in liver disease. Clin Liver Dis 2009; 13(1): 11-20.
20. Koruk M, Onuk MD, Akçay F, Savas MC. Serum thrombopoietin levels in patients with chronic hepatitis and liver cirrhosis, and its relationship with circulating thrombocyte counts. Hepatogastroenterology 2002; 49(48): 1645-8.
21. Freni MA, Spadaro A, Ajello A, et al. Serum thrombopoietin in chronic liver disease: relation to severity of the disease and spleen size. 2002; 49(47): 1382-5.
22. Escolar G, Cases A, Viñas M, et al. Evaluation of acquired platelet dysfunctions in uremic and cirrhotic patients using the platelet function analyzer (PFA-100 ): influence of hematocrit elevation. Haematologica 1999; 84(7): 614-9.
23. Shattil SJ, Anaya-Galindo R, Bennett J, Colman RW, Cooper RA. Platelet hypersensitivity induced by cholesterol incorporation. J Clin Invest 1975; 55(3): 636-43.
24. Watanabe M, Shiraishi K, Itakura M, Matsuzaki S. Relationship between platelet membrane lipid compositions and platelet aggregability in alcoholic liver disease. Alcohol Clin Exp Res 1998; 22(Suppl 3): 97-102. de Boer MT, Christensen MC, Asmussen M, et al. The impact of intraoperative transfusion of platelets and red blood cells on survival after liver transplantation. Anesth Analg 2008; 106(1): 32-44.
25. Boks AL, Brommer EJ, Schalm SW, Van Vliet HH. Hemostasis and fibrinolysis in severe liver failure and their relation to hemorrhage. Hepatology 1986; 6(1): 79-86.
26. Violi F, Ferro D, Basili S, et al. Hyperfibrinolysis increases the risk of gastrointestinal hemorrhage in patients with advanced cirrhosis. Hepatology 1992; 15(4): 672-6.
27. Lisman T, Leebeek FW, Mosnier LO, et al. Thrombin-activatable fibrinolysis inhibitor deficiency in cirrhosis is not associated with increased plasma fibrinolysis. Gastroenterology 2001; 121(1): 131Agarwal S, Joyner KA Jr, Swaim MW. Ascites fluid as a possible origin for hyperfibrinolysis in advanced liver disease. Am J Gastroenterol 2000; 95(11): 3218-24.
28. Piscaglia F, Donati G, Giannini R, Bolondi L. Liver cirrhosis, ascites, and hyperfibrinolysis. Am J Gastroenterol 2001; 96(11): 32
29. Ferro D, Celestini A, Violi F. Hyperfibrinolysis in liver disease. Clin Liver Dis 2009; 13(1): 21-31.
30. Munoz SJ, Stravitz RT, Gabriel DA. Coagulopathy of acute liver failure. Clin Liver Dis 2009; 13(1): 95-107.
31. Youssef WI, Salazar F, Dasarathy S, Beddow T, Mullen KD. Role of fresh frozen plasma infusion in correction of coagulopathy of chronic liver disease: a dual phase study. Am J Gastroenterol 2003; 98(6): 1391-4.
32. Porte RJ. Antifibrinolytics in liver transplantation: they are effective, but what about the risk-benefit ratio? Liver Transpl 2004; 10(2): 285Montalto P, Vlachogiannakos J, Cox DJ, Pastacaldi S, Patch D, Burroughs AK. Bacterial infection in cirrhosis impairs coagulation by a heparin effect: a prospective study. J Hepatol 2002; 37(4): 463101
33. Papatheodoridis GV, Patch D, Webster GJ, Brooker J, Barnes E, Burroughs AK. Infection and hemostasis in decompensated cirrhosis: a prospective study using thrombelastography. Hepatology 1999; 29(4): 1085-90.
34. SPLIT Research Group. Studies of Pediatric Liver Transplantation (SPLIT): year 2000 outcomes. Transplantation 2001; 72(3): 463
35. Najimi M, Sokal E. Update on liver cell transplantation. J Pediatr Gastroenterol Nutr 2004; 39(4): 311-9.
36. Hendrickson RJ, Karrer FM, Wachs ME, Slater K, Bak TE, Kam I. Pediatric liver transplantation. Cur Opinion Pediatr 2004; 16(3): 309Abramson O, Rosenthal P. Current status of pediatric liver transplantation. Clin Liver Dis 2000; 4(3): 533-52.
37. Pugh RN. Pugh’s grading in the classification of liver decompensation. Gut 1992; 33(11): 1583.
38. Schepke M, Roth F, Fimmers R, et al. Comparison of MELD, ChildPugh, and Emory model for the prediction of survival in patients undergoing transjugular intrahepatic portosystemic shunting. Am J Gastroenterol 2003; 98(5): 1167-74.
39. Argo CK, Balogun RA. Blood products, volume control, and renal support in the coagulopathy of liver disease. Clin Liver Dis 2009; 13(1): 73-85.
40. Porte RJ, Caldwell SH. The role of recombinant factor VIIa in liver transplantation. Liver Transpl 2005; 11(8): 872-4.
41. Lisman T, Leebeek FW, Meijer K, Van Der Meer J, Nieuwenhuis HK, De Groot PG. Recombinant factor VIIa improves clot formation but not fibrolytic potential in patients with cirrhosis and during liver transplantation. Hepatology 2002; 35(3): 616-21.
42. Jeffers L, Chalasani N, Balart L, Pyrsopoulos N, Erhardtsen E. Safety and efficacy of recombinant factor VIIa in patients with liver disease undergoing laparoscopic liver biopsy. Gastroenterology 2002; 123(1): 118-26.
43. Shami VM, Caldwell SH, Hespenheide EE, Arseneau KO, Bickston SJ, Macik BG. Recombinant activated factor VII for coagulopathy in fulminant hepatic failure compared with conventional therapy. Liver Transpl 2003; 9(2): 138-43.
44. Bosch J, Thabut D, Bendtsen F, et al. Recombinant factor VIIa for upper gastrointestinal bleeding in patients with cirrhosis: a randomized, double-blind trial. Gastroenterology 2004; 127(4): 1123