Clinical and laboratory evaluation of National Health Service factor VIII concentrate (8Y) for the treatment of von Willebrand's disease

Abstract

This study was carried out to assess the efficacy of NHS 8Y concentrate in the treatment of patients with von Willebrand's disease (vWD). Eight patients (two type I vWD, one type IIA vWD, two type IIB vWD, and three type III vWD) were treated on a total of 10 occasions with 8Y. Following each treatment episode there was a temporary correction of patients' bleeding time (BT) measurements. Other laboratory parameters--von Willebrand factor ristocetin cofactor activity (vWf:RiCo), vWf antigen (vWf:Ag) levels, and factor VIII coagulant activity (factor VIII:C)--were also corrected. Plasma vWf multimers temporarily reflected those present in the infused concentrate. An effective clinical response was observed in each case despite, as revealed by autoradiography and scanning densitometry of SDS-agarose electrophoresis gels, a reduction in the concentration of the largest vWf multimers in 8Y compared with normal plasma. Overall, the clinical effectiveness of 8Y in vWD was comparable to that seen with cryoprecipitate. We conclude that NHS 8Y concentrate may be used as an alternative to cryoprecipitate for the treatment of vWD.

Pharmacokinetics and hemostatic effect of different factor VIII/von Willebrand factor concentrates in von Willebrand's disease type III

“…Hemate-P (Behring) has been reported to contain all multimers of the vWF [4] and to be efficient and safe for the treatment of vWD [1,5,37]. A French concentrate containing all vWF multimers and only a low concentration of FVIII [11,21] and a British FVIII concentrate (8Y) [9,31] have both been reported to shorten the prolonged bleeding time and to be hemostatically effective in vWD.…”

Experience with Haemate P in von Willebrand’s Disease in Adults

“…Reports of successful use of Hae mate P in vWD were also published by Scharrer and Vigh [5], Takahashi et al [6], Czapek et al [7], Fukui et al [1], Vigh et al [8], Berntorp and Nilsson [2], Ieko et al [9], Rose et al [10], Logan [11], Mannucci et al [12], Rodeghiero et al [13], Yoshioka et al [14], and Foster [15], Several other new FVIII concentrates look promising for treatment of vWD. Successful use of SD-FVIII concentrates and other FVIII preparations were reported by Furlan et al [16], Gazengel et al [ 17], Mazurier et al [18], Logan and Higgins [ 19], Pasi et al [20], Palmer et al [21], Cumming et al [22], Lawrie et al [23], Oates et al [24], Retzios et al [25], and Hanna et al [26]. The different concentrates are similarly effective in attaining high levels of FVIII.…”

Replacement Therapy with Virus- Inactivated Plasma Concentrates in von Willebrand Disease

“…Recently, a solventdetergenttreated concentrate containing almost exclusively vWF with little or no FVIII has been prepared by the same commercial manufacturer [36]. Preliminary data show that the concentrate is very efficient in shortening or cor recting the Ivy BT in patients and that FVIII levels are also increased for several hours [36], Finally, full correction of the Ivy BT was reported for 14 patients with various types of vWD after infusion of an intermediate-purity, heattreated concentrate (8Y; Bio Product Laboratory, UK) [37,38]. The multimeric structure of this concentrate is not totally normal, like that of all virus-inactivated con centrates that we tested ( fig.…”

“…Perhaps the BT cannot be normalized in vWD by vWF-containing plasma products because plasma vWF does not exchange with platelet vWF, making it necessary to infuse normal platelets con taining vWF. see 4,10,12,19-21,39; for virus-inactivated concentrates, see [33][34][35]37,38]. BT correction de notes reaching normal values; partial correction, shortening of 50% or more in comparison to basal values; no effect, no shortening or shortening of less than 50% in comparison to preinfusion values.…”