Major surgery for a gastric cancer in a haemophilic with high inhibitor titre successfully performed by the use of recombinant FVIIa

Abstract

A total gastrectomy with omentectomy and resection of the distal oesophagus in a 69-year-old haemophilia A patient with high inhibitor of 128 Bethesda units is described. Surgery was successfully performed after infusion of 112 microg kg-1 bw of recombinant FVIIa. Ninety-two microg kg-1 were given thereafter at time intervals of 2 h until 12 h, then every 3 h until 24 h, and every 4 h until 48 h after surgery. Doses were gradually reduced in the following days and finally discontinued on day 28 after surgery. The complete treatment schedule required the administration of a total of 708 mg of recombinant FVIIa. Using this approach, we observed normal haemostasis, and there were no signs of excessive postoperative bleeding or wound haematoma. No clinical side-effects or evidence of systemic activation of coagulation occurred during the treatment. As judged from the clinical course of this major surgery, recombinant FVIIa appears to be highly efficacious and safe and should be used as first line treatment in high titre inhibitor patients with cross-reactivity to porcine factor VIII, undergoing surgery.

Intestinal surgery with activated recombinant factor VII prophylaxis in patients with haemophilia A and high responding inhibitors

“…During a third herniotomy, covered with two injections of 75 ìg rFVIIa/kg 2 h apart (without antifibrinolytic therapy), slight oozing from the surgical wound was observed 3 h after the second rFVIIa injection; it ceased promptly after a further rFVIIa injection and rFVIIa treatment was continued, but the patient started to bleed profusely from the wound 59 h after the procedure, requiring rescue with FVIII [17]. Three major gastrointestinal procedures with rFVIIa prophylaxis have been reported [12,18]. A patient undergoing total gastrectomy with omentectomy and resection of the distal oesophagus [18] was treated initially with 112 ìg/kg then 92 ìg/kg every 2 h for 12 h before increasing the dosing interval; antifibrinolytic drug therapy was given concomitantly; maelena occurred on day 8 and the haemoglobin level fell; the maelena diminished after a rFVIIa dose increment, but nonetheless persisted for 10 days; no further fall in the haemoglobin level occurred.…”

“…Three major gastrointestinal procedures with rFVIIa prophylaxis have been reported [12,18]. A patient undergoing total gastrectomy with omentectomy and resection of the distal oesophagus [18] was treated initially with 112 ìg/kg then 92 ìg/kg every 2 h for 12 h before increasing the dosing interval; antifibrinolytic drug therapy was given concomitantly; maelena occurred on day 8 and the haemoglobin level fell; the maelena diminished after a rFVIIa dose increment, but nonetheless persisted for 10 days; no further fall in the haemoglobin level occurred. The total dose of rFVIIa was 708 mg and the treatment period was 24 days.…”

“…Few published data are available on rFVIIa prophylaxis during surgery on the central nervous system [10,12] and other types of surgery, such as urologic [8,14], ophthalmologic [10,15], abdominal [16] and intestinal surgery [9,10,14,[17][18][19]. Here we describe the use of rFVIIa prophylaxis during intestinal surgery in five patients with haemophilia A and high responding inhibitors.…”

Critical appraisal of the role of recombinant activated factor VII in the treatment of hemophilia patients with inhibitors

“…The outcome revealed 14 good results, 1 fair result and 2 poor outcomes 55. Furthermore, a case report56–58 and case series59–61 of major surgeries among hemophilia patients with inhibitors using rFVIIa ranging from 90 to 300 μg/kg every 2 hours in the first 24 hours followed by 90 to 120 μg/kg with prolonged intervals of 3 to 6 hours have gradually been reported, with effective response rates ranging from 66% to 100%.…”

Factor VIIa: Use in Patients With Hemophilia

“…Rare surgical interventions involving the use of rFVIIa in hemophilic inhibitor patients include major surgery for gastric cancer, 15 resection of a massive pseudotumour, 16 and heart transplantation 17 in a 14 year-old patient who had suffered a myocardial infarction and subsequent cardiomyopathy following use of aPCC. …”