The thrombogram in rare inherited coagulation disorders: Its relation to clinical bleeding

Thrombosis and Haemostasis

Volumn 88, Issue 4, 2002, Pages 576-582

  Al Dieri, Raed ^a   Peyvandi, Flora ^a   Santagostino, Elena ^a   Giansily, Muriel ^a   Mannucci, Pier Mannuccio ^a   Schved, Jean François ^a   Béguin, Suzette ^a   Hemker, H Coenraad ^a  

^a MAASTRICHT UNIVERSITY   (Netherlands)

Author keywords

Clotting factors; Thrombin; Thrombogram

Indexed keywords

BLOOD CLOTTING FACTOR 10; BLOOD CLOTTING FACTOR 11; BLOOD CLOTTING FACTOR 12; BLOOD CLOTTING FACTOR 5; BLOOD CLOTTING FACTOR 7; PROTHROMBIN; THROMBIN; CHROMOGENIC SUBSTRATE;

AREA UNDER THE CURVE; ARTICLE; BLEEDING; BLEEDING TENDENCY; BLOOD CLOTTING; BLOOD CLOTTING DISORDER; BLOOD CLOTTING FACTOR 10 DEFICIENCY; BLOOD CLOTTING FACTOR 11 DEFICIENCY; BLOOD CLOTTING FACTOR 12 DEFICIENCY; BLOOD CLOTTING FACTOR 5 DEFICIENCY; BLOOD CLOTTING FACTOR 7 DEFICIENCY; CONCENTRATION (PARAMETERS); DISEASE ASSOCIATION; DISEASE SEVERITY; GENETIC DISORDER; HUMAN; MAJOR CLINICAL STUDY; PRIORITY JOURNAL; PROTEIN BLOOD LEVEL; PROTHROMBIN DEFICIENCY; THROMBIN TIME; BLOOD; BLOOD CLOTTING TEST; HOSPITALIZATION; KINETICS; METABOLISM; PREDICTION AND FORECASTING;

BLOOD COAGULATION TESTS; CHROMOGENIC COMPOUNDS; COAGULATION PROTEIN DISORDERS; HEMORRHAGE; HUMANS; KINETICS; PREDICTIVE VALUE OF TESTS; PROTHROMBIN; SEVERITY OF ILLNESS INDEX; THROMBIN;

EID: 0036797888     PISSN: 03406245     EISSN: None     Source Type: Journal    
DOI: 10.1055/s-0037-1613258     Document Type: Article

References (23)

1. Peyvandi F, Mannucci PM. Rare coagulation disorders. Thromb Haemost 1999; 82: 1207-14.
2. Peyvandi F, Mannucci PM, Asti D, Abdoullahi M, Di Rocco N, Sharifian R. Clinical manifestations in 28 Italian patients with severe factor VII deficiency. Hemophilia 1997; 3: 242-6.
3. Lak M, Sharifian R, Peyvandi F, Mannucci PM. Symptoms of inherited factor V deficiency in 35 Iranian patients. Br J Haematol 1998; 103: 1067-9.
4. Peyvandi F, Mannucci PM, Lak M, Abdoullahi M, Zeinali S, Sharifian R, Perry D. Congenital factor X deficiency: spectrum of bleeding symptoms in 32 Iranian patients. Br J Haematol 1998; 102: 626-8.
5. Lak M, Keihani M, Elahi F, Peyvandi F, Mannucci PM. Bleeding and thrombosis in 55 patients with inherited afibrinogenaemia. Br J Haematol 1999; 107: 204-6.
6. Duga S, Asselta R, Santagostino E, Zeinali S, Simonic T, Malcovati M, Mannucci PM, Tenchini ML. Missense mutations in the human beta fibrinogen gene cause congenital afibrinogenemia by impairing fibrinogen secretion. Blood 2000; 95: 1336-41.
7. Peyvandi F, Jenkins PV, Mannucci PM, Billio A, Zeinali S, Perkins SJ, Perry DJ. Molecular characterisation and three-dimensional structural analysis of mutations in 21 unrelated families with inherited factor VII deficiency. Thromb Haemost 2000; 84: 250-7.
8. Akhavan S, Mannucci PM, Lak M, Mancuso G, Mazzucconi MG, Rocino A, Jenkins PV, Perkins SJ. Identification and three-dimensional structural analysis of nine novel mutations in patients with prothrombin deficiency. Thromb Haemost 2000; 84: 989-97.
9. Montefusco MC, Duga S, Asselta R, Santagostino E, Mancuso G, Malcovati M, Mannucci PM, Tenchini ML. A novel two base pair deletion in the factor V gene associated with severe factor V deficiency. Br J Haematol 2000; 111: 1240-6.
10. van Wijk R, Montefusco MC, Duga S, Asselta R, van Solinge W, Malcovati M, Tenchini ML, Mannucci PM. Coexistence of a novel homozygous nonsense mutation in exon 13 of the factor V gene with the homozygous Leiden mutation in two unrelated patients with severe factor V deficiency. Br J Haematol 2001; 114: 871-4.
11. Peyvandi F, Carew JA, Perry DJ, Hunault M, Khanduri U, Perkins SJ, Mannucci PM, Bauer KA. Abnormal secretion and function of recombinant human factor VII as the result of modification to a calcium binding site caused by a 15-base pair insertion in the F7 gene. Blood 2001; 97: 960-5.
12. Rijkers DT, Wielders SJ, Tesser GI, Hemker HC. Design and synthesis of thrombin substrates with modified kinetic parameters. Thromb Res 1995; 79: 491-9.
13. Rosing J, Tans G, Govers-Riemslag JW, Zwaal RF, Hemker HC. The role of phospholipids and factor Va in the prothrombinase complex. J Biol Chem 1980; 255: 274-83.
14. Hendrix H, Lindhout T, Mertens K, Engels W, Hemker HC. Activation of human prothrombin by stoichiometric levels of staphylocoagulase. J Biol Chem 1983; 258: 3637-44.
15. Hemker HC, Willems GM, Béguin S. A computer assisted method to obtain the prothrombin activation velocity in whole plasma independent of thrombin decay processes. Thromb Haemost 1986; 56: 9-17.
16. Hemker HC, Wielders S, Kessels H, Béguin S. Continuous registration of thrombin generation in plasma, its use for the determination of the thrombin potential. Thromb Haemost 1993; 70: 617-24
17. Hemker HC, Giesen PL, Ramjee M, Wagenvoord R, Béguin S. The thrombogram: monitoring thrombin generation in platelet-rich plasma. Thromb Haemost 2000; 83: 589-91.
18. Wielders S, Mukherjee M, Michiels J, Rijkers DT, Cambus JP, Knebel RW, Kakkar V, Hemker HC, Béguin S. The routine determination of the endogenous thrombin potential, first results in different forms of hyper- and hypocoagulability. Thromb Haemost 1997; 77: 629-36.
19. Keularts IM, Zivelin A, Seligsohn U, Hemker HC, Béguin S. The role of factor XI in thrombin generation induced by low concentrations of tissue factor. Thromb Haemost 2001; 85: 1060-5.
20. Hemker HC, Béguin S. Phenotyping the clotting system. Thromb Haemost 2000; 84: 747-51.
21. Azar AJ, Koudstaal PJ, Wintzen AR, van Bergen PF, Jonker JJ, Deckers JW. Risk of stroke during long-term anticoagulant therapy in patients after myocardial infarction. Ann Neurol 1996; 39: 301-7.
22. Béguin S, Lindhout T, Hemker HC. The mode of action of heparin in plasma. Thromb Haemost 1988; 60: 457-62.
23. Butenas S, van't Veer C, Mann KG. "Normal" thrombin generation. Blood 1999; 94: 2169-78.