Morfini M, Mannucci P M, Tenconi P M, Longo G, Mazzucconi M G, Rodeghiero F, Ciavarella N, De Rosa V, Arter A
Hematology Department, University of Florence, Italy.
Thromb Haemost. 1993 Aug 2;70(2):270-2.
A monoclonally-purified factor VIII (FVIII) concentrate, containing little von Willebrand factor (vWF), was infused to 11 patients with severe von Willebrand disease and unmeasurable levels of plasma vWF. In comparison with the historical data obtained infusing hemophiliacs in the same conditions, monoclonally-purified FVIII had a significantly shorter half-life and faster clearance from plasma but similar in vivo recovery and volume of distribution. Two additional patients with severe von Willebrand disease were also infused with recombinant FVIII totally devoid of vWF. Half-life was very short and in vivo recovery low, with a larger volume of distribution than for monoclonally-purified FVIII. We conclude that in patients with severe von Willebrand disease the small amounts of vWF contained in the monoclonally-purified FVIII concentrate are not sufficient to stabilize infused FVIII, nor to support the normal circulation of endogenous FVIII that these patients produce at a normal rate.
将一种单克隆纯化的因子VIII（FVIII）浓缩物（含少量血管性血友病因子（vWF））输注给11例患有严重血管性血友病且血浆vWF水平无法测量的患者。与在相同条件下输注血友病患者所获得的历史数据相比，单克隆纯化的FVIII半衰期明显较短，从血浆中清除更快，但体内回收率和分布容积相似。另外两名患有严重血管性血友病的患者也输注了完全不含vWF的重组FVIII。半衰期非常短，体内回收率低，分布容积比单克隆纯化的FVIII大。我们得出结论，在患有严重血管性血友病的患者中，单克隆纯化的FVIII浓缩物中所含少量vWF不足以稳定输注的FVIII，也不足以支持这些患者以正常速率产生的内源性FVIII的正常循环。
