Anti-inhibitor Coagulant Complex (Autoplex) for treatment of factor VIII inhibitors in hemophilia

Abstract

Fourteen individuals with severe hemophilia complicated by factor VIII inhibitors (1 to 132 Bethesda Units) were treated for 33 bleeding episodes with a new activated prothrombin complex concentrate, Anti- Inhibitor Coagulant Complex (Autoplex, Hyland, Glendale, Calif.). Excellent or good results were observed in 21 of 25 minor bleeding episodes treated, which included joint, soft tissue, and mucous membrane hemorrhages. Eight major bleeding problems (an epidural bleed, a puncture wound, 2 serious soft tissue hemorrhages, 2 lacerations, and 2 major surgical procedures) were treated with excellent (6) or good (2) results. No serious complications were encountered, but two children developed transient hypofibrinogenemia following Autoplex infusion. Although some shortening of the prothrombin time and activated partial thromboplastin time was noted after infusion of Autoplex, there is no useful laboratory test for monitoring therapy. Despite the unknown mechanism of action for bypassing factor VIII, Autoplex appears to be a useful and needed interim product and is safe and effective. In view of the possible potentiation of thrombosis concurrent use of fibrinolytic inhibitors should be avoided.

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

“…By cross-checking relevant articles, additional articles were added to the list in a non-standardized manner. Almost 60 articles were identified describing 332 EOS procedures [7,41,[43][44][45][46][47][48][49][50][51][52][53][54][55][56][57][58][60][61][62][63][64][65][66][67][68][69][73][74][75]. Based on the classification of Perez Bianco et al [70] in which synoviorthesis and arthrocentesis are classified as minor and all other procedures are classified as major, 210 (63%) of the procedures were major and 122 (37%) were minor EOS procedures.…”

“…In addition, results from a recent cost-benefit analysis suggest that major knee surgery using rFVIIa in haemophilia patients with inhibitors may be costeffective because of the reduced number of bleedings or improved quality of life experienced by these individuals following surgery [59]. Pd-activated prothrombin complex concentrates and pd-PCCs have also been used to cover surgical procedures in haemophilia patients with inhibitors, although experience is largely based on minor EOS procedures [20][21][22]30,32,34,46,49,[60][61][62][63][64][65][66][67][68][69]. While most case studies revealed positive outcomes with rFVIIa and pd-APCC, the small numbers of patients and potential selective reporting may bias results.…”

Management of haemophilia in patients with high‐titre inhibitors: focus on the evolution of activated prothrombin complex concentrate AUTOPLEX® T

“…Although no serious complications were noted, two children developed reversible hypo®brinogenaemia immediately following infusion [1]. Therefore, Abildgaard concluded that anti®brinolytics should not be used in conjunction with AUTOPLEX 1 to minimize the remote possibility of a thrombotic event [1], although it has been used safely and eectively by other haemophilia treaters [16]. However, it has been hypothesized that the small amounts of heparin in AUTOPLEX 1 may provide protection against thrombosis [7].…”

Surgery in haemophilia patients with inhibitors, with special emphasis on orthopaedics: Madrid experience

“…Two approaches for the treatment of patients with inhibi-tors have been proposed. Immune tolerance induction using high-dose FVIII or FIX daily or twice daily for a period of a few months to several years may completely eliminate the inhibitor, again allowing the patient to be treated efficiently with FVIII or FIX [1,2]. However, immune tolerance induction fails in around 20% of cases and is not proposed for all patients because of the high probability of failure or adverse events.…”