Current Trend of Invasive Orthopaedic Interventions for People with Haemophilia in Romania: Single Centre Experience

Abstract

Objective In countries with low factor concentrate consumption, disabling joint disease remains the major morbidity in patients with haemophilia. The objective of the present analysis is to express the trend and profile of invasive orthopaedic interventions in our country with low usage of factor replacement, lacking the prophylaxis program until recent years.

Patients and Methods This retrospective descriptive study was conducted in our university centre in Timisoara with long-lasting experience in haemophilia care, which succeeded in developing an exceptionally valuable genuine comprehensive inter-institutional cooperation. This study refers to 115 invasive interventions performed on 97 patients: 83 with haemophilia A, 10 with haemophilia B and 4 with von Willebrand disease in the period of 2001 to 2017; 17 of them had inhibitors, 5 developing inhibitors after the intervention.

Results and Discussion The cohort of patients consisted mostly of young adults, aged between 7 and 49 years. The vast majority of them, 91.3% of the patients, had the burden of severe chronic arthropathy. This was the reason for 95 interventions, with programmed solution. In 20 cases the cause of invasive interventions was emergency. Knee and hip replacement represented 28% of the major interventions. The complications we encountered were excessive bleeding (12.2%), infection (13%) and inhibitor development (4.3%).

Conclusion Surgery is a demanding intervention in patients with haemophilia, which unfortunately cannot be ignored in our country. Hopefully, the improved availability and accessibility to replacement therapy will eliminate the necessity of these interventions, optimizing the outcomes for the next generations.

Keywords

haemophilia A - haemophilia B - surgery - orthopaedics

• References

• 1 Oldenburg J. Optimal treatment strategies for hemophilia: achievements and limitations of current prophylactic regimens. Blood 2015; 125 (13) 2038-2044
• 2 Makris M. Prophylaxis in haemophilia should be life-long. Blood Transfus 2012; 10 (02) 165-168
• 3 Giangrande PLF, Peyvandi F, O'Mahony B. , et al. Kreuth IV: European consensus proposals for treatment of haemophilia with coagulation factor concentrates. Haemophilia 2017; 23 (03) 370-375
• 4 Schramm W, Gringeri A, Ljung R. , et al; ESCHQOL Study Group. Haemophilia care in Europe: the ESCHQoL study. Haemophilia 2012; 18 (05) 729-737
• 5 Coppola A, Morfini M, Cimino E, Tufano A, Cerbone AM, Di Minno G. Current and evolving features in the clinical management of haemophilia. Blood Transfus 2014; 12 (Suppl. 03) s554-s562
• 6 Solimeno LP, Escobar MA, Krassova S, Seremetis S. Major and minor classifications for surgery in people with hemophilia: a literature review. Clin Appl Thromb Hemost 2018; 24 (04) 549-559
• 7 Srivastava A, Brewer AK, Mauser-Bunschoten EP. , et al; Treatment Guidelines Working Group on Behalf of The World Federation Of Hemophilia. Guidelines for the management of hemophilia. Haemophilia 2013; 19 (01) e1-e47
• 8 Wiedel J, Stabler S, Geraghty S, Funk S. Joint replacement surgery in haemophilia. World Federation Haemophilia 2010; 50: 1-17
• 9 Tobase P, Lane H, Siddiqi AE. , et al; Hemophilia Treatment Center Network (HTCN) Study Investigators. Risk factors associated with invasive orthopaedic interventions in males with haemophilia enrolled in the Universal Data Collection program from 2000 to 2010. Haemophilia 2018; 24 (06) 964-970
• 10 Tobase P, Lane H, Siddiqi AE, Ingram-Rich R, Ward RS. ; Universal Data Collection Joint Outcome Working Group, Hemophilia Treatment Center Network (HTCN) Study Investigators. Declining trends in invasive orthopedic interventions for people with hemophilia enrolled in the Universal Data Collection program (2000–2010). Haemophilia 2016; 22 (04) 604-614
• 11 de Kleijn P, Sluiter D, Vogely HCh, Lindeman E, Fischer K. Long-term outcome of multiple joint procedures in haemophilia. Haemophilia 2014; 20 (02) 276-281
• 12 Schild FJA, Mauser-Bunschoten EP, Verbout AJ, Van Rinsum AC, Roosendaal G. Total knee arthroplasty in hemophilic arthropathy: efficiency of clotting factor usage in multijoint procedures. J Thromb Haemost 2009; 7 (10) 1741-1743
• 13 De Kleijn P, Fischer K, Vogely HCh, Hendriks C, Lindeman E. In-hospital rehabilitation after multiple joint procedures of the lower extremities in haemophilia patients: clinical guidelines for physical therapists. Haemophilia 2011; 17 (06) 971-978
• 14 Rodriguez-Merchan EC, Gomez-Cardero P, Jimenez-Yuste V. Infection after total knee arthroplasty in haemophilic arthropathy with special emphasis on late infection. Haemophilia 2011; 17 (05) e831-e832
• 15 Kotela A, Wilk-Frańczuk M, Żbikowski P, Łęgosz P, Ambroziak P, Kotela I. Revision knee arthroplasty in patients with inherited bleeding disorders: a single-center experience. Med Sci Monit 2017; 23: 129-137
• 16 Rodríguez-Merchán EC. Total knee arthroplasty in hemophilic arthropathy. Am J Orthop 2015; 44 (12) E503-E507
• 17 Iorio A, Barbara AM, Makris M. , et al. Natural history and clinical characteristics of inhibitors in previously treated haemophilia A patients: a case series. Haemophilia 2017; 23 (02) 255-263
• 18 Habermann B, Ewald W, Rauschmann M, Zichner L, Kurth AA. Fracture of ceramic heads in total hip replacement. Arch Orthop Trauma Surg 2006; 126 (07) 464-470
• 19 Habermann B, Eberhardt C, Hovy L, Zichner L, Scharrer I, Kurth AA. Total hip replacement in patients with severe bleeding disorders. A 30 years single center experience. Int Orthop 2007; 31 (01) 17-21
• 20 Colvin BT, Astermark J, Fischer K. , et al; Inter Disciplinary Working Group. European principles of haemophilia care. Haemophilia 2008; 14 (02) 361-374