| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
* From the Faculty of Medicine, University of British Columbia; the
Division of Hematology and Oncology, the
Department of Anesthesia, and the
Department of Orthopedic Surgery, British Columbia Children’s Hospital, Vancouver, British Columbia, Canada.
Address correspondence to: Dr. Robert Purdy, Staff Anesthesiologist, Department of Pediatric Anesthesia, Room 1L7, British Columbia’s Children’s Hospital, 4480 Oak Street, Vancouver, British Columbia V6H 3V4, Canada. E-mail: rpurdy{at}cw.bc.ca
Purpose: To describe the successful perioperative hemostatic management of a Jehovah’s Witness patient with hemophilia B and anaphylactic inhibitors to factor IX, undergoing scoliosis surgery.
Clinical features: A 14 
-yr-old boy with severe hemophilia B who had a history of anaphylactic inhibitors to factor IX was scheduled to undergo corrective scoliosis surgery. He was initially started on epoetin alfa and iron supplementation to maximize preoperative red cell mass. Additionally, he was placed on a desensitization protocol of recombinant coagulation factor IX (rFIX) and was then treated with activated recombinant coagulation factor VII (rFVIIa) during the postoperative period. Tranexamic acid was given concomitantly. The intraoperative blood loss was approximately 350 mL. The nadir hemoglobin concentration was 111 g·L–1 on postoperative days one and two. On postoperative day 11, the patient was stable and discharged home with a hemoglobin of 138 g·L–1. He did not require blood transfusion and no adverse events were observed.
Conclusions: The use of rFIX, rFVIIa, erythropoetin, iron, and tranexamic acid before, during and after scoliosis surgery may be a viable and safe option for hemophilia patients with inhibitors, who refuse blood products.
1 Lee JW. Von Willebrand disease, hemophilia A and B, and other factor deficiencies. Int Anesthesiol Clin 2004; 42: 59–76.[Medline]
2 Lusher JM. Inhibitor antibodies to factor VIII and factor IX: management. Semin Thromb Hemost 2000; 26: 179–88.[Medline]
3 Quintana-Molina M, Martinez-Bahamonde F, Gonzalez-Garcia E, et al. Surgery in haemophilic patients with inhibitor: 20 years of experience. Haemophilia 2004; 10 Suppl 2: 30–40.
4 Martlew VJ. Peri-operative management of patients with coagulation disorders. Br J Anaesth 2000; 85: 446–55.
5 Rodriguez-Merchan EC, Rocino A. Literature review of surgery management in inhibitor patients. Haemophilia 2004; 10 Suppl 2: 22–9.[Medline]
6 Poon MC. Use of recombinant factor VIIa in hereditary bleeding disorders. Curr Opin Hematol 2001; 8: 312–8.[Medline]
7 Hedner U, Glazer S, Pingel K, et al. Successful use of recombinant factor VIIa in patient with severe haemophilia A during synovectomy. Lancet 1988; 2: 1193.[Medline]
8 Roberts HR, Monroe DM, White GC. The use of recombinant factor VIIa in the treatment of bleeding disorders. Blood 2004; 104: 3858–64.
9 Dioun AF, Ewenstein BM, Geha RS, Schneider LC. IgE-mediated allergy and desensitization to factor IX in hemophilia B. J Allergy Clin Immunol 1998; 102: 113–7[Medline]
10 Sieger L, Aledort L. Inhibitor challenges in the paediatric setting. Haemophilia 2006; 12: 106–7.[Medline]
11 Pellegrino E, Thomasma DC. For The Patient’s Good: The Restoration of Beneficence in Health Care. Oxford University Press; 1988.
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
