Rationale for the use of high dose rFVIIa in a high‐titre inhibitor patient with haemophilia B during major orthopaedic procedures

Haemophilia - Tập 7 Số 5 - Trang 517-522 - 2001
Herbert Cooper1, Connie P. Jones1, Edmund Campion1, Barbara A. Konkle1, Ulla Hedner2
1Department of Paediatrics, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA
2Department of Medicine and Orthopaedics, University of Lund, Wallenberg Research Laboratory, University Hospital of Malmö, Sweden

Tóm tắt

Inhibitor development is a serious complication in patients with haemophilia A and B. Historically, a lack of optimal therapies and factor products for treating inhibitor patients resulted in many patients developing chronic haemophilic arthropathies and flexion contractures of the involved joints. The introduction of immune‐tolerance protocols to eradicate high‐titre inhibitors has greatly diminished the incidence of these types of complications but as in the case reported here, immune tolerance is not always successful. Various elective surgical procedures were often delayed or not even considered in patients with inhibitor because of the variability in achieving adequate haemostasis and the thrombotic risks involved with the use of activated prothrombin‐complex concentrates (APCCs) over extended periods of time. The development of recombinant factor VIIa (rFVIIa; NovoSevenTM) and its demonstrated safety and efficacy in treating inhibitor patients has opened new possibilities for addressing severe arthropathy with flexion contracture. This case report demonstrates that the use of rFVIIa in such a situation must include dosing flexibility that is both patient‐specific and related to the potential for bleeding; the ability to maintain clinical haemostasis with a prophylactic dose of rFVIIa given as little as once daily; and the capacity for higher doses of rFVIIa, particularly in children because their kinetic profiles differ from adults.

Từ khóa


Tài liệu tham khảo

10.1016/S0955-3886(98)00026-5

10.1097/00001721-199803000-00002

10.1055/s-0037-1615357

10.1111/j.1365-2796.1992.tb00546.x

10.1046/j.1365-2796.1997.130135000.x

10.1111/j.1600-0609.1993.tb00638.x

Pabinger I, 1987, Home treatment of haemophilia, Wein Klin Wochenschr, 99, 773

Jones P., 1992, Haemophilia home therapy, Haemostasis, 22, 247

HilgartenMW CardiD GoldbergI.Home Therapy. In: Forbes CD Aledort L Madhok R eds.HaemophiliaLondon: Chapman & Hall Medical. 1997: 171–81.

10.1055/s-0037-1615388

Nilsson IM, 1981, A procedure for removing high titre antibodies by extracorporeal protein‐A‐sepharose in a patient with haemophilia B and antibodies, Blood, 58, 38, 10.1182/blood.V58.1.38.38

10.1056/NEJM198804143181503

GlaznerS&HednerUet al.Clinical update on the use of recombinant factor VIIa. In: Aledort LM Hoyer LW Lusher JM Reisner HM White GC II eds.Inhibitors to Coagulation FactorsNew York: Plenum Press. 1995: 163–74.

HednerU KristensenHI BerntorpE LjungR PetriniP TengbornL.Pharmacokinetics of rFVIIa in Children. World Federation of Haemophilia Conference Den Hague The Netherlands 1998 (Abstract).

10.1038/clpt.1994.80

10.1046/j.1365-2141.1997.4463256.x

Kjalke M, 1998, The effects of activated factor VII in a cell‐based model for tissue factor‐initiated coagulation, Blood Coagul Fibrinolysis, 9, 21

KjalkeM MonroeDM HoffmanM RobertsHR HednerU EzbanM.High‐dose factor VIIa restores platelet activation and total thrombin generation in a model system mimicking hemophilia A or B conditions.Thromb Haemost Suppl1999;August:303–303.

10.3109/ort.1965.36.suppl-77.01

Wildgoose P, 1992, Measurement of basal levels of factor VIIa in haemophilia A and B patients, Blood, 80, 25, 10.1182/blood.V80.1.25.25

10.1016/0049-3848(94)90227-5

Van'T Veer C, 2000, Inhibition of thrombin generation by the zymogen factor VII: implications for the treatment of haemophilia A by Factor VIIa, Blood, 95, 1330, 10.1182/blood.V95.4.1330.004k28_1330_1335

Thông tin
Thông tin xuất bản

Haemophilia

Tập 7 Số 5

517-522

Thông tin tác giả