Cases of surgery in high‐responder haemophilia patients

Goudemand, Jenny; Tagariello, Giuseppe; Lopaciuk, F.

doi:10.1111/j.1365-2516.2004.00940.x

Cited by 27 publications

(32 citation statements)

References 2 publications

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“…The cases covered by Tagariello in 2000 were considered in Goudemand et al Work published in 1996 by Ingerslev et al has been followed up and expanded in 2002 . Rodriguez‐Merchan et al have sequentially expanded upon their experience with publications in 2003, 2007 and 2010 …”

Section: Methodsmentioning

confidence: 99%

Over two decades of orthopaedic surgery in patients with inhibitors—Quantifying the complication of bleeding

et al. 2018

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Patients with haemophilia who have developed inhibitors against factor VIII (FVIII) or factor IX present a significant concern to those surgeons who operate on them. The evidence base for bypassing agents such as recombinant factor VIIa and activated prothrombin complex concentrate has amassed over several decades. The literature is open to positive interpretation on the successful use of these agents in the treatment of inhibitor‐positive patients. However, there are equally persistent concerns amongst surgeons, in particular orthopaedic surgeons, regarding the high complication rate of bleeding. To explore and quantify this concern, we present a literature review spanning two decades of publications on haemophilia patients with inhibitors undergoing orthopaedic surgery. Irrespective of the progress made with haemostatic protocols, trepidation on embarking on surgery is valid. The high risk of bleeding is a function of the inherent complexity of the disease and rightfully translates into difficulties in its management. Combined with the prospect of orthopaedic surgery, those involved in the care of such patients are justified in their continued anxiety and diligence when considering the benefits in quality of life against the prevalent complications.

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Section: Methodsmentioning

confidence: 99%

Over two decades of orthopaedic surgery in patients with inhibitors—Quantifying the complication of bleeding

et al. 2018

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“…However, the majority of data on the use of rFVIIa are derived from case studies. A number of case studies have reported on the successful use of rFVIIa as haemostatic cover during elective surgery in haemophilia patients with high‐titer inhibitors [7,8,10–58]. In addition, results from a recent cost–benefit analysis suggest that major knee surgery using rFVIIa in haemophilia patients with inhibitors may be cost‐effective because of the reduced number of bleedings or improved quality of life experienced by these individuals following surgery [59].…”

Section: Introductionmentioning

confidence: 99%

Elective orthopaedic surgery for haemophilia patients with inhibitors: single centre experience of 40 procedures and review of the literature

et al. 2011

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With the introduction of safe and effective factor VIII/IX-bypassing agents--recombinant activated factor VII (rFVIIa) and plasma-derived activated prothrombin complex concentrates (pd-APCC)--elective orthopaedic surgery (EOS) is a viable option for haemophilia patients with inhibitors. We report a series of patients with haemophilia and inhibitors undergoing EOS between 1997 and 2008 using bypassing agents to provide haemostatic cover. All inhibitor patients undergoing EOS and receiving rFVIIa, plasma-derived prothrombin complex concentrates (pd-PCC) or pd-APCC as haemostatic cover were included. Patients were operated on by the same surgeon and were managed by the same haemophilia treatment centre. Forty procedures (25 minor and 15 major) were conducted in 18 patients. Twenty-one minor cases were covered using rFVIIa, three with pd-PCC, and one with pd-APCC; all major cases were covered using rFVIIa. Bleeding was no greater than expected compared with a non-haemophilic population in all 25 minor procedures. In the major procedure group, there was no excessive bleeding in 40% of cases (6/15) and bleeding completely stopped in response to rFVIIa. For the remaining nine cases, bleeding response to rFVIIa was described as 'markedly decreased' or 'decreased' in 4/15 cases and 'unchanged' in 5/15 cases. Overall, efficacy of rFVIIa, based on final patient outcome, was 85%. One death occurred as a result of sepsis secondary to necrotizing fasciitis. Good control of haemostasis can be achieved with bypassing agents in haemophilia patients with inhibitors undergoing minor EOS; rFVIIa was used as an effective bypassing agent, enabling EOS in patients undergoing minor and major procedures.

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“…Topical application of fibrin sealant during the intraoperative period to minimize capillary ooze and the use of vasoconstrictors and antifibrinolytics can be used to enhance the effect of rVIIa and improve haemostasis (Schulman 1998). Activated prothrombin complex concentrate (aPCC) has also been used to cover surgical procedures in haemophilia patients with inhibitors (Dimichele 2006;Goudemand 2004;Hvid 2002;Negrier 1997;Tjonnfjord 2004) and data suggests similar efficacy to rVIIa, albeit having the risk of an anamnestic rise in antibody titre (Schulman 1998), a risk not present with rVIIa. Current guidelines give equal merit to rVIIa and aPCC in managing bleeding in haemophilia (Hay 2006).…”

Section: Recombinant Factor Viiamentioning

confidence: 99%