Medical management of patients with hemophilia A, B and Von Willebrand disease who underwent surgery a sixteen year experience at Hospital de San José, Bogotá
Objetivo: describir el manejo médico de tipo hemostático durante el pre, trans y posoperatorio de pacientes con hemofilia y enfermedad de von Willebrand. Materiales y métodos: serie de casos de pacientes diagnosticados con hemofilia A, B, enfermedad de vo
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Peña Castellanos, Ángela M., Solano Trujillo, M. H., & Villamizar Gómez, L. (2011). Medical management of patients with hemophilia A, B and Von Willebrand disease who underwent surgery a sixteen year experience at Hospital de San José, Bogotá. Journal of Medicine and Surgery Repertoire, 20(4), 233–239. https://doi.org/10.31260/RepertMedCir.v20.n4.2011.779
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Abstract
Objective: to describe haemostatic management of patients with hemophilia and von Willebrand disease before, during and after surgery. Materials and Methods: case series of patients diagnosed with hemophilia A, B, von Willebrand disease and factor VII deficiency, from January 1993 to June 2009 at Hospital de San José, Bogotá DC. Results: 35 scheduled surgeries of various specialties in 28 patients (4 females, 24 males). For those with hemophilia A and B the objective in terms of level of haemostasis in orthopedic procedures was: on day one 100%, from day two to five: 74% and day 6 and so on: 60%; in dental procedures, on day one the factor was corrected an average of 85% and from day two to five: 65%. Patients with Von Willebrand disease were managed with a 50 IU/k dose every 8 to 24 hours. There were 3 cases of infection and no deaths occurred. Conclusions: Hospital de San José has gained a vast experience in conducting scheduled surgical procedures in patients with hemophilia A, B and von Willebrand disease. These patients have been managed successfully during these 16 years with a Iow complication rate.
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23. Rodeghiero F, Castaman G, Tosetto A. How I treat von Willebrand disease. Blood. 2009;114(6):1158-65.
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2. Lichtman M, Beutler E, J(jpps T. Seligsohn U, Kaushanshy K,Prchal J. Williams Hematology. 7a. edición. 2006.
3. Mariani G, Konk.le B, lngerslev J. Congenital factor VIl deficiency: therapy with recombinan! activated factor Vll-a critica! appraisal. Hemophi!ia. 2006;12:19-27.
4. Lapecorella M,Mariani G. Factor VII deficiency: defining the clinical picture and optimizing therapeutic options. Haemophilia 2008; 1 4:1170-5.
5. lngerslev J, Hvad J. Surgery in Hemophilia. The general view: Patient selection, liming and preoperative assessment. Semin Hematol. 2006;43(Suppl 1):23-6.
6. Rickard K. Guidelines for therapy and optima! dosages of coagulation factors for treatment of bleeding and surgery in haemophilia. Haemophilia. 1995;1(Suppl. 1):8-13.
7. FederaciónMundial de Hemofilia. Directrices para el tratamiento de la hemofilia. 2005.
8. Comisión latinoamericana sobre la terapéutica de personas con inhibidores CLOTTING. 2010.
9. World Federation of Hemophilia Global Survey. Annual global survey. 2009.
10. Mathews V, ViswabandyaA, Baidya S,George B,NairS,ChandyM,eta!. Surgery for hemophilia in developing countries. Semin Thromb Hemost. 2005;31(5):538-43.
11. Plug J, Van Der Bom G, Peter M, Mauser-Bunschoten EP, De Goede A, Bolder L, et al. Mortality and causes of death in patients with hemophilia, 1992-2001: a prospective cohort study. J Thromb Haemost. 2006;4(3):510-6.
12. Yee T, Lee C. Transfusion-transmitted infection in hemophilia in developing countries. Seminars in thrombosis and hemostasis.2005;31(5):527-37.
13. Bolton-Maggs P. Optima! haemophilia care versus reality. British Journal of Hematology. 2005;132:671-82.
14. Astermark J. Why do inhibitors develop? Principies of and factors influencing the risk for inhibitor development in haemophilia. Haemophilia. 2006;(12):52-60.
15. Bemtorp E. Options for treating acote bleeds in addition to bypassing agents: extracorporealimmunoadsorption, FVIII/FIX, desmopressinandantifibrinolytics. Haemophilia. 2006;12(Suppl 6):62-6.
16. Kraut EH, Aledort LM, Arkin S, Stine KC, Wong WY. SurgicaJ interventions in a cohort of patients with haemophilia A and inhibitors: an experiential retrospective chart review. Haemophilia. 2007;13:508-17.
17. Giangrande PL, Wilde JT, Madan B, Ludlam CA, Tuddenham EG, Goddard NJ, et al. Consensos protocol for lhe use of recombinantactivatedfactor VII (eptacog alta (activated); NovoSeven) in elective orthopaedic surgery in haemophilic patients with inhibitors. Haemophilia. 2009;15:501-8.
18. Marrnuci P. How I treal age-related morbidilies in elderly persons with hemophilia. Blood. 2009;114:5256-63.
19. Hermans C, Altisent C, Batorova A, Chambos! H, De Moerloose P, Karafoulidou A, et al. Replacement therapy for invasive procedures in patients with haemophilia: literature review, European Surveyand recommendations. Haemophilia. 2009;15:639- 58.
20. Franchini M, Rossetti G, Tagliaferri A, Pattacini C, Pozzoli D, Lorenz C, et al. Dental procedures in adult patients with hereditary bleeding disorders: JO years experience in lhree Italian Hemophilia Centers. Haemophilia. 2005 Sep;11(5):504-9.
21. Gómez JF, Duarte M, Raffansanabria F. Radical prostatectomy in hemophiliac patienl. Arch Esp Uro!. 2003 Sep;56(7):827-9.
22. Giangrande P, Wilde J. Consensos protocol for the use of recombinan! activated factor Vlla in elective orthopaedic surgery in haemophilic patients with inhibitors. Hemophilia. 2009;15:501-8.
23. Rodeghiero F, Castaman G, Tosetto A. How I treat von Willebrand disease. Blood. 2009;114(6):1158-65.
24. Schulman S, Loogna J, Wallensten R. Minimizing factor requirements for surgery wilhout increased risk. Haemophilia. 2004 Oct;I0(Suppl 4):35-40.
25. Srivastava A, You S, Ayob Y, Chuansumrit A, De Bosch N, Pérez-Bianco R, et al. Hemophilia treatment in developing countries: products and protocols. Seminars in thrombosis and hemostasis. 2005;31(495):537.