Criterios de respuesta para definir éxito en la terapia de pacientes hemofílicos llevados a cirugía: Revisión ampliada de la literatura (RAL)

Response criteria to define success in the therapy of hemophilic patients undergoing surgery: Extended literature review (RAL)

La hemofilia, enfermedad congénita de baja prevalencia en la población general, se clasifica en tipos A y B según su deficiencia sea del factor VIII o IX de la coagulación. Desde hace más de 40 años existen preparados plasmáticos de cada uno de los factores y en la actualidad contamos con derivados recombinantes. Los pacientes que requieren intervenciones quirúrgicas son un reto para los diferentes grupos de manejo, porque el estándar de criterios para definir la respuesta a la terapia no está claro. Se realizó esta revisión ampliada de la literatura con el fin de evaluar cuáles son los criterios de respuesta en las diferentes publicaciones. En las principales bases de datos médicos, se recolectaron y tabularon 61 artículos después de aplicar los criterios de inclusión y exclusión, encontrando que múltiples estudios aplicaban diferentes parámetros para evaluar el sangrado como son los niveles de factor de la coagulación, tromboelastografía, tiempo de generación de trombina, reintervención quirúrgica, duración de la hospitalización y evaluación de dolor, con lo cual concluimos que hay heterogeneidad en los parámetros que aplican los autores en relación con los objetivos principal y secundarios de nuestra revisión.

1. Aledort LM. History of haemophilia. Haemophilia. 2007 Dec;13 Suppl 5:1-2.

2. Oldenburg J, Dolan G, Lemm G. Haemophilia care then, now and in the future. Haemophilia. 2009 Jan; 15 Suppl 1:2-7.

3. Stachnik J. Hemophilia: Etiology, complications, and current options in management. Clin Pharm. 2010: 45.

4. Wong T, Recht M. Current options and new developments in the treatment of haemophilia. Drugs. 2011 Feb; 71(3):305-20.

5. Lee CA. The best of times, the worst of times: a story of haemophilia. Clin Med. 2009 Oct; 9(5):453-8.

6. Evatt BL. The tragic history of AIDS in the hemophilia population. 1982-1984. J Thromb Haemost. 2006 Nov; 4(11):2295-301.

7. Mannucci PM. AIDS, hepatitis and hemophilia in the 1980s: memoirs from an insider. J Thromb Haemost. 2003 Oct;1(10):2065-9

8. Chorba TL, Holman RC, Strine TW, Clarke MJ, Evatt BL. Changes in longevity and causes of death among persons with hemophilia A. Am J Hematol. 1994 Feb;45(2):112-21

9. Mannucci PM, Tuddenham EG. The hemophilias from royal genes to gene therapy. N Engl J Med. 2001 Jun 7; 344(23):1773-9.

10. Rodriguez-Merchan EC. Local fibrin glue and chitosan-based dressings in haemophilia surgery. Blood Coagul Fibrinolysis. 2012 Sep; 23(6):473-6.

11. Aznar JA, Marco A, Jiménez-Yuste V, et al. Is on-demand treatment effective in patients with severe haemophilia?. Haemophilia. 2012 Sep;18(5):738-42.

12. Eckhardt CL, Mauser-Bunschoten EP, Peters M, Leebeek FW, et al. Inhibitor incidence after intensive FVIII replacement for surgery in mild and moderate haemophilia A: a prospective national study in the Netherlands. Br J Haematol. 2012 Jun; 157(6):747-52.

13. Batorova A, Holme P, Gringeri A, Richards M, Hermans C, Altisent C, et al. Continuous infusion in haemophilia: current practice in Europe. Haemophilia. 2012 Sep; 18(5):753-9.

14. Pérez-Garrido R, Alonso N, Jiménez-Yuste V et al. Efficacy of factor IX Grifols(®) in surgery: experience of an international multicenter retrospective study. Haemophilia. 2012 Sep; 18(5):e372-3.

15. Holmström M, Tran HT, Holme PA. Combined treatment with APCC (FEIBA®) and tranexamic acid in patients with haemophilia A with inhibitors and in patients with acquired haemophilia A--a two-centre experience. Haemophilia. 2012 Jul; 18(4):544-9.

16. Coppola A, Franchini M, Makris M, Santagostino E, et al. Thrombotic adverse events to coagulation factor concentrates for treatment of patients with haemophilia and von Willebrand disease: a systematic review of prospective studies. Haemophilia. 2012 May;18(3):e173-87.

17. Berntorp E, Astermark J, Baghaei F, et al. Treatment of haemophilia A and B and von Willebrand’s disease: summary and conclusions of a systematic review as part of a Swedish health-technology assessment. Haemophilia. 2012 Mar; 18(2):158-65.

18. Zülfikar B, Aydogan G, Salcioglu Z, Oner AF, Kavakli K, et al. Efficacy of FEIBA for acute bleeding and surgical haemostasis in haemophilia A patients with inhibitors: a multicentre registry in Turkey. Haemophilia. 2012 May; 18(3):383-91.

19. Eckhardt CL, van der Bom JG, van der Naald M, et al. Surgery and inhibitor development in hemophilia A: a systematic review. J Thromb Haemost. 2011 Oct; 9(10):1948-58.

20. Rossi M, Jayaram R, Sayeed R. Do patients with haemophilia undergoing cardiac surgery have good surgical outcomes?. Interact Cardiovasc Thorac Surg. 2011 Sep; 13(3):320-31.

21. Mauser-Bunschoten EP, Kleine Budde I, Lopaciuk S, Koopman MM, et al. An ultrapure plasma-derived monoclonal antibody-purified factor IX concentrate (Nonafact®), results of phase III and IV clinical studies. Haemophilia. 2011 May; 17(3):439-45.

22. Oldenburg J, Goudemand J, Valentino L, et al. Postauthorization safety surveillance of ADVATE [antihaemophilic factor (recombinant), plasma/albumin-free method] demonstrates efficacy, safety and low-risk for immunogenicity in routine clinical practice. Haemophilia. 2010 Nov; 16(6):866-77.

23. Windyga J, Rusen L, Gruppo R, et al. BDDrFVIII (Moroctocog alfa [AF-CC]) for surgical haemostasis in patients with haemophilia A: results of a pivotal study. Haemophilia. 2010 Sep 1; 16(5):731-9.

24. Martinowitz U, Luboshitz J, Bashari D, et al. Stability, efficacy, and safety of continuously infused sucrose-formulated recombinant factor VIII (rFVIII-FS) during surgery in patients with severe haemophilia. Haemophilia. 2009 May; 15(3):676-85.

25. Giangrande PL, Wilde JT, Madan B, et al. Consensus protocol for the use of recombinant activated factor VII [eptacog alfa (activated); NovoSeven] in elective orthopaedic surgery in haemophilic patients with inhibitors. Haemophilia. 2009 Mar; 15(2):501-8.

26. Négrier C, Shapiro A, Berntorp E, et al. Surgical evaluation of a recombinant factor VIII prepared using a plasma/albumin-free method: efficacy and safety of Advate in previously treated patients. Thromb Haemost. 2008 Aug; 100(2):217-23.

27. Schwarz KB, Zellos A, Stamato L, et al. Percutaneous liver biopsy in hemophiliac children with chronic hepatitis C virus infection. J Pediatr Gastroenterol Nutr. 2008 Apr; 46(4):423-8.

28. Miles J, Rodríguez-Merchán EC, Goddard NJ. The impact of haemophilia on the success of total hip arthroplasty. Haemophilia. 2008 Jan; 14(1):81-4.

29. Correa ME, Annicchino-Bizzacchi JM, Jorge J Jr, et al. Clinical impact of oral health indexes in dental extraction of hemophilic patients. J Oral Maxillofac Surg. 2006 May; 64(5):785-8.

30. Franchini M, Rossetti G, Tagliaferri A, et al. Dental procedures in adult patients with hereditary bleeding disorders: 10 years experience in three Italian Hemophilia Centers. Haemophilia. 2005 Sep; 11(5):504-9.

31. Türkmen C, Zülflkar B, Taşer O, et al. Radiosynovectomy in hemophilic synovitis: correlation of therapeutic response and blood-pool changes. Cancer Biother Radiopharm. 2005 Jun; 20(3):363-70.

32. Soroa VE, del Huerto Velázquez Espeche M, Giannone C, et al. Effects of radiosynovectomy with p-32 colloid therapy in hemophilia and rheumatoid arthritis. Cancer Biother Radiopharm. 2005 Jun; 20(3):344-8.

33. Lee AP, Boyle CA, Savidge GF, Fiske J. Effectiveness in controlling haemorrhage after dental scaling in people with haemophilia by using tranexamic acid mouthwash. Br Dent J. 2005 Jan 8; 198(1):33-8.

34. Karaman MI, Zulfikar B, Caskurlu T, Ergenekon E. Circumcision in hemophilia: a cost-effective method using a novel device. J Pediatr Surg. 2004 Oct; 39(10):1562-4.

35. Stieltjes N, Altisent C, Auerswald G, et al. Continuous infusion of B-domain deleted recombinant factor VIII (ReFacto) in patients with haemophilia A undergoing surgery: clinical experience. Haemophilia. 2004 Sep; 10(5):452-8.

36. Rodriguez-Merchan EC, Wiedel JD, Wallny T, et al. Elective orthopaedic surgery for inhibitor patients. Haemophilia. 2003 Sep; 9(5):625-31.

37. Hoots WK, Leissinger C, Stabler S, et al. Continuous intravenous infusion of a plasma-derived factor IX concentrate (Mononine) in haemophilia B. Haemophilia. 2003 Mar; 9(2):164-72.

38. Giangrande PL; KOGENATE Bayer Study Group. Safety and efficacy of KOGENATE Bayer in previously untreated patients (PUPs) and minimally treated patients (MTPs). Haemophilia. 2002 Mar; 8 Suppl 2:19-22.

39. Ragni MV, Pasi KJ, White GC, Giangrande PL, et al. Use of recombinant factor IX in subjects with haemophilia B undergoing surgery. Haemophilia. 2002 Mar; 8(2):91-7.

40. Smith OP. Recombinant factor VIIa in the management of surgery and acute bleeding episodes in children with haemophilia and high-responding inhibitors. Pathophysiol Haemost Thromb. 2002; 32 Suppl 1:22-5.

41. Scharrer I, Brackmann HH, Sultan Y, et al. Efficacy of a sucrose-formulated recombinant factor VIII used for 22 surgical procedures in patients with severe haemophilia A. Haemophilia. 2000 Nov; 6(6):614-8.

42. MacKinlay N, Taper J, Renisson F, et al. Cardiac surgery and catheterization in patients with haemophilia. Haemophilia. 2000 Mar;6(2):84-8.

43. Bollard CM, Teague LR, Berry EW, Ockelford PA. The use of central venous catheters (portacaths) in children with haemophilia. Haemophilia. 2000 Mar;6(2):66-70.

44. Tagariello G, Davoli PG, Gajo GB, et al. Safety and efficacy of high-purity concentrates in haemophiliac patients undergoing surgery by continuous infusion. Haemophilia. 1999 Nov; 5(6):426-30.

45. Scharrer I. Recombinant factor VIIa for patients with inhibitors to factor VIII or IX or factor VII deficiency. Haemophilia. 1999 Jul; 5(4):253-9.

46. Rochat C, McFadyen ML, Schwyzer R, et al. Continuous infusion of intermediate-purity factor VIII in haemophilia A patients undergoing elective surgery. Haemophilia. 1999 May; 5(3):181-6.

47. Shapiro AD, Gilchrist GS, Hoots WK, et al. Prospective, randomised trial of two doses of rFVIIa (NovoSeven) in haemophilia patients with inhibitors undergoing surgery. Thromb Haemost. 1998 Nov; 80(5):773-8.

48. Négrier C, Menart C, Attali O, et al. Evaluation of coagulation equilibrium at baseline and during factor VIII and factor IX replacement in haemophiliacs. Blood Coagul Fibrinolysis. 1998 Mar; 9 Suppl 1:S135-41.

49. Santagostino E, Gringeri A, Muça-Perja M, et al. A prospective clinical trial of implantable central venous access in children with haemophilia. Br J Haematol. 1998 Sep; 102(5):1224-8.

50. F Gordon, P Mistry, C Sabin, and C Lee. Outcome of orthotopic liver transplantation in patients with haemophilia. Gut. May 1998; 42(5): 744-9.

51. Djulbegovic B, Marasa M, Pesto A, et al. Safety and efficacy of purified factor IX concentrate and antifibrinolytic agents for dental extractions in hemophilia B. Am J Hematol. 1996 Feb; 51(2):168-70.

52. Waly NG. Local antifibrinolytic treatment with tranexamic acid in hemophilic children undergoing dental extractions. Egypt Dent J. 1995 Jan;41(1):961-8.

53. Pearce MS, Smith MA, Savidge GF. Supramalleolar tibial osteotomy for haemophilic arthropathy of the ankle. J Bone Joint Surg Br. 1994 Nov; 76(6):947-50.

54. Santagostino E, Mannucci PM, Gringeri A, et al. Markers of hypercoagulability in patients with hemophilia B given repeated, large doses of factor IX concentrates during and after surgery. Thromb Haemost. 1994 Jun; 71(6):737-40.

55. Goldsmith JC, Kasper CK, Blatt PM, et al. Coagulation factor IX: successful surgical experience with a purified factor IX concentrate. Am J Hematol. 1992 Jul;40(3):210-5.

56. Erken EH. Radiocolloids in the management of hemophilic arthropathy in children and adolescents. Clin Orthop Relat Res. 1991 Mar; (264):129-35.

57. Forbes CD, Barr RD, Reid G, Thomson C, et al. Tranexamic acid in control of haemorrhage after dental extraction in haemophilia and Christmas disease. Br Med J. 1972; 2(5809): 311-3.

58. Simpson E, Lin Y, Stanworth S, et al. Recombinant factor VIIa for the prevention and treatment of bleeding in patients without haemophilia. Cochrane Database Syst Rev. 2012 Mar 14;3: CD005011.

59. Scharrer I. Recombinant factor VIIa for patients with inhibitors to factor VIII or IX or factor VII deficiency. Haemophilia. 1999 Jul; 5(4):253-9.

60. Négrier C, Menart C, Attali O, et al. Evaluation of coagulation equilibrium at baseline and during factor VIII and factor IX replacement in haemophiliacs. Blood Coagul Fibrinolysis. 1998 Mar; 9 Suppl 1:S135-41.

61. Shapiro AD, Gilchrist GS, Hoots WK, et al. Prospective, randomised trial of two doses of rFVIIa (NovoSeven) in haemophilia patients with inhibitors undergoing surgery. Thromb Haemost. 1998 Nov; 80(5):773-8.