Patients with hereditary hemostatic disorders, characterized by a tendency to bleeding or thrombosis, constitute a serious challenge in the dental practice. Advances in the medical diagnosis of hemostatic disorders have exposed dental professionals to new patients not amenable to the application of the management protocols associated with other, more well-known, disorders. It is the aim of this paper to review the evidence, to highlight the areas of major concern, and to suggest management regimens for patients with hereditary hemostatic disorders. An extensive review has been made (PubMed, Science Direct, Web of Knowledge, etc.) of literature pertaining to hereditary disorders affecting blood coagulation factors and how they affect the practice of dentistry. Several aspects relating to the care of such patients must be recognized and taken into consideration when dental treatment is planned. Replacement of deficient coagulation factors ensures that safe dental treatment will be carried out. However, the half-life of such coagulation factors requires that dental treatment be specifically planned and adapted to the type of pathology involved.
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References
1.
Alcalay M, Deplas A -2002-. Rheumatological management of patients with hemophilia. Part 1: joint manifestations. Joint Bone Spine69:442–449.
2.
Awasthy N, Aggarwal KC, Gupta H, Saluja S -2004-. Congenital hypofibrinogenemia. Indian Pediatr41:185–187.
3.
Badurowa A, Galazka Z, Sciborski R -1983-. Oral surgery in a patient with congenital factor V deficiency. Wiad Lek36:1797–1800.
4.
Bayry J, Lacroix-Desmazes S, Pashov A, Stahl D, Hoebeke J, Kazatchkine MD, et al. -2003-. Autoantibodies to factor VIII with catalytic activity. Autoimmun Rev2:30–35.
5.
Blanchard N, Jeanjean P, Lepointe F, Dieval J, Ossart M -1996-. Factor XI deficiency, a new way of substitution: human purified concentrates. Ann Fr Anesth Reanim15:1207–1210.
6.
Brenner B -2000-. Hereditary deficiency of vitamin K-dependent coagulation factors. Thromb Haemost84:935–936.
7.
Brewer AK, Roebuck EM, Donachie M, Hazard A, Gordon K, Fung D, et al. -2003-. The dental management of adult patients with haemophilia and other congenital bleeding disorders. Haemophilia9:673–677.
8.
Cattaneo M -1997-. Review of clinical experience of desmopressin in patients with congenital and acquired bleeding disorders. Eur J Anaesthesiol Suppl14:10–14.
9.
Cillo J, Pulsipher A, Rutherford CJ, Ellis E 3rd -2001-. Third molar extractions in a patient with congenital afibrinogenemia: a case report. J Oral Maxillofac Surg59:935–936.
10.
Crean SJ, Sivarajasingam V, Muhammed J, Sharma V, Fardy M -2000-. Thrombophilia and dental surgery: a report of dental extraction in a patient with protein S deficiency. Dent Update27:302–305.
11.
Cutando A, Gil JA -1999-. The dental patient with hemostatic disorders. A review of hemostasia physiopathology for dental professionals. Med Oral4:485–493.
12.
Dahlback B -2003-. The discovery of activated protein C resistance. J Thromb Haemost1:3–9.
13.
Daly HM, Haddon ME -1988-. Clinical experience with a Pasteurised human plasma concentrate in factor XIII deficiency. Thromb Haemost59:171–174.
14.
Deering SH, Landy HJ, Tchabo N, Kessler C -2003-. Hypodysfibrinogenemia during pregnancy, labor, and delivery. Obstet Gynecol101:1092–1094.
15.
del Rey López C, Alonso Chico A -2002-. Antithrombin III deficiency and regional anesthesia. Rev Esp Anestesiol Reanim49:117–118.
16.
Deloughery TG -1999-. Hemostasis and thrombosis. Georgetown, TX: Landes Publishing.
17.
Di Paola J, Nugent D, Young G -2001-. Current therapy for rare factor deficiencies. Haemophilia7-Suppl 1-:16–22.
18.
Diethorn ML, Weld LM -1989-. Physiologic mechanisms of hemostasis and fibrinolysis. J Cardiovasc Nurs4:1–10.
19.
Divanon F, Hecquard C, Borel-Derlon A -2002-. Experience with use of recombinant activated factor VII. J Clin Pharm Ther27:133–138.
20.
Djulbegovic B, Marasa M, Pesto A, Kushner GM, Hadley T, Joseph G, et al. -1996-. Safety and efficacy of purified factor IX concentrate and antifibrinolytic agents for dental extractions in hemophilia B. Am J Hematol51:168–170.
21.
Egbring R, Kroniger A, Seitz R -1996-. Factor XIII deficiency: pathogenic mechanisms and clinical significance. Semin Thromb Hemost22:419–425.
22.
Eskandari N, Feldman N, Greenspan JS -2002-. Factor VII deficiency in pregnancy treated with recombinant factor VIIa. Obstet Gynecol99:935–937.
23.
Favier R, Aoki N, de Moerloose P -2001-. Congenital alpha-2--plasmin inhibitor deficiencies: a review. Br J Haematol114:4–10.
24.
Franchini M, Capra F, Tagliaferri A, Rossetti G, De Gironcoli M, Rocca P, et al. -2002-. Update on chronic hepatitis C in hemophiliacs. Haematologica87:542–549.
25.
François P, Verstraeten L, Dinant JP -1989-. The physiology of hemostasis: plasma and tissue factors in coagulation and fibrinolysis. J Pharm Belg44:308–314.
26.
Gatti L, Carnelli V, Rusconi R, Moia M -2003-. Heparin-induced thrombocytopenia and warfarin-induced skin necrosis in a child with severe protein C deficiency: successful treatment with dermatan sulfate and protein C concentrate. J Thromb Haemost1:387–388.
27.
Giangrande PL -2003-. Treatment of patients with haemophilia and inhibitory antibodies. Indian J Pediatr70:655–659.
28.
Girolami A, Scarano L, Saggiorato G, Girolami B, Bertomoro A, Marchiori A -1998-. Congenital deficiencies and abnormalities of prothrombin. Blood Coagul Fibrinolysis9:557–569.
Harper K, Friedland ML -1992-. Hageman factor deficiency presentation and implications for management. Conn Med56:469–471.
31.
Harrington B -2000-. Primary dental care of patients with haemophilia. Haemophilia6-Suppl 1-:7–12.
32.
Kasper CK -2000-. Hereditary plasma clotting factor disorders and their management. Haemophilia6-Suppl 1-:13–27.
33.
Kehrel BE -2003-. Blood platelets: biochemistry and physiology. Hamostaseologie23:149–158.
34.
Knight RD, Barr CF, Alving BM -1985-. Replacement therapy for congenital Factor X deficiency. Transfusion25:78–80.
35.
Kobayashi T, Terao T, Kojima T, Takamatsu J, Kamiya T, Saito H -1990-. Congenital factor XIII deficiency with treatment of factor XIII concentrate and normal vaginal delivery. Gynecol Obstet Invest29:235–238.
36.
Kouides PA, Kulzer L -2001-. Prophylactic treatment of severe factor X deficiency with prothrombin complex concentrate. Haemophilia7:220–223.
37.
Larson CE, Chang JL, Bleyaert AL, Bedger R -1980-. Anesthetic considerations for the oral surgery patient with hemophilia. J Oral Surg38:516–519.
38.
Lee C, Dusheiko G -2002-. The natural history and antiviral treatment of hepatitis C in haemophilia. Haemophilia8:322–329.
39.
Leissinger CA -1999-. Use of prothrombin complex concentrates and activated prothrombin complex concentrates as prophylactic therapy in haemophilia patients with inhibitors. Haemophilia5-Suppl 3-:25–32.
40.
Little JW, Falace DA, Miller CS, Rhodus NL -1997-. Dental management of the medically compromised patient. 5th ed. St. Louis: CV Mosby Co.
41.
Mannucci PM -1998-. Treatment of von Willebrand disease. Haemophilia4:661–664.
42.
McDonough RJ, Nelson CL -1989-. Clinical implications of factor XII deficiency. Oral Surg Oral Med Oral Pathol68:264–266.
43.
Merchant RH, Agarwal BR, Currimbhoy Z, Pherwani A, Avasthi B -1992-. Congenital factor XIII deficiency. Indian Pediatr29:831–836.
44.
Meyer D, Pietu G, Fressinaud E, Girma JP -1991-. von Willebrand factor: structure and function. Mayo Clin Proc66:516–523.
45.
Montiel-Manzano G, de la Pena-Diaz A, Majluf-Cruz A -2003-. National evaluation of the diagnosis of activated protein C resistance. Rev Invest Clin55:358–369.
46.
Morfini M, Lee M, Messori A -1991-. The design and analysis of half-life and recovery studies for factor VIII and factor IX. Factor VIII/Factor IX Scientific and Standardization Committee of the International Society for Thrombosis and Haemostasis. Thromb Haemost66:384–386.
47.
Mousallem M, Spronk HM, Sacy R, Hakime N, Soute BA -2001-. Congenital combined deficiencies of all vitamin K-dependent coagulation factors. Thromb Haemost86:1334–1336.
48.
Nakahara M, Koyama T -2003-. Congenital plasmin inhibitor deficiencies: basic concepts and clinical applications. Rinsho Ketsueki44:149–158.
49.
Narayanan S, Hamasaki N -1998-. Current concepts of coagulation and fibrinolysis. Adv Clin Chem33:133–168.
50.
Neerman-Arbez M -2001-. The molecular basis of inherited afibrinogenaemia. Thromb Haemost86:154–163.
51.
Nilsson IM -1987-. Coagulation and fibrinolysis. Scand J Gastroenterol Suppl137:11–18.
52.
Norris LA -2003-. Blood coagulation. Best Pract Res Clin Obstet Gynaecol17:369–383.
53.
Okamoto T, Minami K -2003-. Anesthesia for a child with a congenital antithrombin deficiency -abstract-. Can J Anaesth50:311.
54.
Pescatore SL -2001-. Clinical management of protein C deficiency. Expert Opin Pharmacother2:431–439.
55.
Piot B, Sigaud-Fiks M, Huet P, Fressinaud E, Trossaert M, Mercier J -2002-. Management of dental extractions in patients with bleeding disorders. Oral Surg Oral Med Oral Pathol Oral Radiol Endod93:247–250.
56.
Rand ML, Leung R, Packham MA -2003-. Platelet function assays. Transfus Apheresis Sci28:307–317.
Sallah AS, Angchaisuksiri P, Roberts HR -1996-. Use of plasma exchange in hereditary deficiency of factor V and factor VIII. Am J Hematol52:229–230.
59.
Schneppenheim R, Budde U -2004-. Classification of von Willebrand disease. Hamostaseologie24:27–36.
60.
Scully C, Cawson RA -2004-. Medical problems in dentistry. 5th ed. London: Butterworth-Heinemann.
61.
Scully C, Diz Dios P, Giangrande P, Lee C -2002-. Oral health care in hemophilia and other bleeding tendencies. World Federation on Hemophilia. Treatment of Hemophilia Monograph Series. No. 27. UK: The Faculty of General Dental Practice.
62.
Sindet-Pedersen S, Stenbjerg S, Ingerslev J -1988-. Control of gingival hemorrhage in hemophilic patients by inhibition of fibrinolysis with tranexamic acid. J Periodontal Res23:72–74.
63.
Stubbs M, Lloyd J -2001-. A protocol for the dental management of von Willebrand’s disease, haemophilia A and haemophilia B. Aust Dent J46:37–40.
64.
Sun WY, Witte DP, Degen JL, Colbert MC, Burkart MC, Holmback K, et al. -1998-. Prothrombin deficiency results in embryonic and neonatal lethality in mice. Proc Natl Acad Sci USA95:7597–7602.
65.
Takahashi H -1993-. Biosynthesis in the vascular endothelial cells, molecular structure and function of von Willebrand factor. Nippon Rinsho51:1635–1642.
66.
Troy GC -1988-. An overview of hemostasis. Vet Clin North Am Small Anim Pract18:5–20.
67.
Tuddenham EG, Pemberton S, Cooper DN -1995-. Inherited factor VII deficiency: genetics and molecular pathology. Thromb Haemost74:313–321.
68.
Uprichard J, Perry DJ -2002-. Factor X deficiency. Blood Rev16:97–110.
69.
Wilde JT -2004-. HIV and HCV coinfection in haemophilia. Haemophilia10:1–8.
70.
Yamanaka Y, Takeuchi K, Sugimoto M, Sato A, Nakago S, Maruo T -2003-. Dysfibrinogenemia during pregnancy treated successfully with fibrinogen. Acta Obstet Gynecol Scand82:972–973.
71.
Zanon E, Martinelli F, Bacci C, Zerbinati P, Girolami A -2000-. Proposal of a standard approach to dental extraction in haemophilia patients. A case-control study with good results. Haemophilia6:533–536.
