Consensual guidelines of the Czech National Haemophilia Programme (CNHP) for replacement treatment of patients with haemophilia during invasive procedures and surgery, 2nd edition, year 2020

Czech version

Authors: R. Hrdličková ¹; P. Smejkal ²; J. Blatný ³; Z. Hajšmanová ⁴; A. Hluší ⁵; E. Zápotocká ⁶; M. Penka ²; B. Blažek ⁷; Z. Čermáková ¹; Z. Černá ⁸; P. Ďulíček ⁹; J. Hak ¹⁰; D. Pospíšilová ¹¹; D. Procházková ¹²; P. Timr ¹³; J. Ullrychová ¹⁴; I. Vonke ¹⁵; L. Walterová ¹⁶
Authors‘ workplace: Krevní centrum a Klinika hematoonkologie, Fakultní nemocnice Ostrava ¹; Oddělení klinické hematologie, Fakultní nemocnice Brno ²; Oddělení dětské hematologie, Fakultní nemocnice Brno ³; Ústav klinické biochemie a hematologie, Fakultní nemocnice Plzeň ⁴; Hemato-onkologická klinika, Fakultní nemocnice Olomouc ⁵; Klinika dětské hematologie a onkologie, Fakultní nemocnice v Motole, Praha ⁶; Klinika dětského lékařství, Fakultní nemocnice Ostrava ⁷; Dětská klinika, Fakultní nemocnice Plzeň ⁸; IV. Interní hematologická klinika, Fakultní nemocnice Hradec Králové ⁹; Dětská klinika, Fakultní nemocnice Hradec Králové ¹⁰; Dětská klinika, Fakultní nemocnice Olomouc ¹¹; Dětská klinika, Krajská zdravotní a. s., Masarykova nemocnice v Ústí nad Labem ¹²; Dětské oddělení, Nemocnice České Budějovice ¹³; Oddělení klinické hematologie, Krajská zdravotní a. s., Masarykova nemocnice v Ústí nad Labem ¹⁴; Oddělení klinické hematologie, Nemocnice České Budějovice ¹⁵; Oddělení klinické hematologie, Krajská nemocnice Liberec ¹⁶
Published in: Transfuze Hematol. dnes,26, 2020, No. 3, p. 257-262.
Category: Best Practices

Overview

Haemophilia A and B are rare congenital bleeding disorders. Invasive procedures and surgery can be performed safely in patients with haemophilia without inhibitors due to the availability of coagulation factors VIII and IX. The aim of these guidelines is to summarize experience and recommendations for treatment including optimal factors VIII/IX levels during surgery, laboratory monitoring and replacement treatment duration after these procedures. A special attention is drawn to the procedures in patiens being treated by the bispecific monoclonal antibody emicizumab.

Keywords:

haemophilia – surgery – factor VIII – factor IX – emicizumab – replacement treatment

Sources

1. Hrdličková R, Smejkal P, Blatný J, et al. Konsenzuální doporučení Českého národního hemofilického programu (ČNHP) pro zajištění chirurgických a invazivních výkonů u pacientů s hemofilií. Transfuze Hematol. Dnes. 2016;22(1):39–43.

2. Guidelines for the Management of Hemophilia. World Federation of Hemophilia 2012; publikováno elektronicky 6. června 2012. DOI: 10.1111/j.1365-2516.2012.02909.

3. Australian Haemophilia Centre Directors´ Organisation (AHCDO). Guideline for the management of patients with haemophilia undergoing surgical procedures. November 2010. https://www.ahcdo. org.au/documents/item/13.

4. Bátorová A, Jankovičová D, Žarnovičanová M, et al. Národné štandardné postupy pre liečbu hemofilie a iných vrodených koagulopatií v Slovenskej republike. Lek Obz. 2008; 56: 330–340.

5. Hermans C, Altisent C, Batorova A, et al. Replacement therapy for invasive procedures in patients with haemophilia: literature review, European survey and recommendation. Haemophilia. 2009;15:639–658.

6. Ljung RC, Knobe K. How to manage invasive procedures in children with haemophilia. Br J Haematol. 2012;157:519–528.

7. Pruthi RK. Laboratory monitoring of new hemostatic agents for hemophilia. Semin Hematol. 2016;53:28–44.

8. Gray E, Kitchen S, Bowyer A, et al. Laboratory measurement of factor replacement therapies in the treatment of congenital haemophilia: A United Kingdom Haemophilia Centre Doctors‘ Organisation guideline. Haemophilia. 2020;26(1):6–16.

9. Wallny TA, Strauss AC, Goldmann G, et al. Elective total knee arthroplasty in haemophilic patients. Proposal for a clinical pathway. Hamostaseologie. 2014;34(Suppl 1): S23–S29.

10. Wiedel J, Stabler S, Geraghty S, Funk S. Joint replacement surgery in hemophilia. World Federation of Hemophilia. 2010. http://www1. wfh.org/publication/files/pdf-1210.pdf

11. Batorova A, Holme P, Gringeri A, et al. Continuous infusion in haemophilia: current practice in Europe. Haemophilia. 2012;18(5):753–759.

12. Holme PA, Tjønnfjord GE, Batorova A. Continuous infusion of coagulation factor concentrates during intensive treatment. Haemophilia. 2018;24:24–32.

13. Bidlingmaier C, Demi MM, Kurnik K. Continuous infusion of factor concentrates in children with haemophilia A in comparison with bolus injections. Haemophilia. 2006;12:212–217.

14. Collins PW, Fischer K, Morfini M, et al. Implications of coagulation factor VIII and IX pharmacokinetics in the prophylactic treatment of haemophilia. Haemophilia. 2011;17:2–10.

15. Australian Haemophilia Centre Directors’Organisation (AHCDO). A consensus statement on the dental treatment of patients with inherited bleeding disorders July 2010. https://www.ahcdo.org.au/ documents/item/14

16. Anderson JAM, Brewer A, Creagh D, et al. Guidance on the dental management of patients with haemophilia and congenital bleeding disorders. Br Dent J. 2013;215:497–504.

17. Susen S, Gruel Y, Godier A, et al. Management of bleeding and invasive procedures in haemophilia A patients with inhibitor treated with emicizumab (Hemlibra®): Proposals from the French network on inherited bleeding disorders (MHEMO), the French Reference Centre on Haemophilia, in collaboration with the French Working Group on Perioperative Haemostasis (GIHP). Haemophilia. 2019;00:1–7.

18. MASAC document #258. Recommendation on the use and management of emicizumab- KXWH (HEMLIBRA®) for hemophilia A with and without inhibitors. https://www.hemophilia.org/sites/ default/files/document/files/258_emicizumab.pdf

19. Levy GG, Asikanius E, Kuebler P, et al. Safety analysis of rFVIIa with emicizumab dosing in congenital hemophilia A with inhibitors: Experience from the HAVEN clinical program. J Thromb Haemost. 2019;17(9):1470–1477.

20. Ozelo MC. Surgery in patients with hemophilia: is thromboprophylaxis mandatory? Thromb Res. 2012;130(Suppl 1):S23–S26.

21. Zakarija A, Aledort L. How we treat: venous trombembolism prevention in haemophilia patients undergoing major orthopaedics surgery. Haemophilia. 2009;15:1337–1338.

22. Hermans C. Venous thromboembolic disease in patients with haemophilia. Thromb Res. 2012;130(Suppl 1):S50-S52.

23. Tintillier V, Branche J, Maunoury V, et al. Colonoscopy in patients with haemophilia: the duration of factor coverage must be adjusted to suit the procedure. Haemophilia. 2013;19:e296–e323.

24. Fogarty PF, Koudide P. How we manage prostate biopsy and prostate cancer therapy in men with haemophilia. Haemophilia. 2012;18:e88–e90.