Santagostino Elena, Mancuso Maria Elisa, Morfini Massimo, Schiavoni Mario, Tagliaferri Annarita, Barillari Giovanni, Mannucci Pier Mannuccio
Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Department of Medicine and Medical Specialities, University of Milan, RCCS Maggiore Hospital, Mangiagalli and Regina Elena Foundation, Italy.
Haematologica. 2006 May;91(5):634-9.
This open-label multicenter trial of solvent/detergent (SD) plasma involving 17 patients with recessively inherited coagulation disorders (one afibrinogenemia, four FV, six combined FV and FVIII, one FX and five FXI deficiencies) evaluated the pharmacokinetics of the deficient factors and hemostatic efficacy.
In vivo recovery (IVR) of the deficient coagulation factor was determined in a non-bleeding state in all patients and the mean values for FV, FVIII, FX, FXI and fibrinogen were 1.3, 1.2, 1.5, 1.3 and 1.5 dL/Kg, respectively. The mean plasma half-life of FV, FVIII and FX was 18, 43 and 33 hours, respectively. All patients underwent replacement therapy for elective procedures at risk of bleeding (surgery in 14 cases and vaginal delivery in two patients), except one treated for a central nervous system surgical emergency.
Treatment courses with SD plasma were judged fully effective in 13/16 cases (81%). In the remaining three cases, mild bleeding occurred after major surgery in a FV deficient patient with a factor level of 43% and in a FXI deficient patient when factor levels were between 20% and 41%; and after minor surgery in a patient with FV and FVIII deficiency when factor levels were 41% and 18%, respectively. Bleeding was controlled by continuing or increasing treatment with SD plasma.
These results suggest that, even though the current absolute risk of blood-borne infections associated with fresh-frozen plasma is relatively small, SD plasma should be preferred in patients with recessively inherited coagulation disorders who need replacement therapy when virus-inactivated single-factor concentrates are not available.
这项关于溶剂/去污剂（SD）血浆的开放标签多中心试验纳入了17例隐性遗传性凝血障碍患者（1例无纤维蛋白原血症、4例FV缺乏、6例FV和FVIII联合缺乏、1例FX缺乏以及5例FXI缺乏），评估了缺乏因子的药代动力学及止血效果。
在所有患者非出血状态下测定缺乏凝血因子的体内回收率（IVR），FV、FVIII、FX、FXI和纤维蛋白原的平均值分别为1.3、1.2、1.5、1.3和1.5 dL/Kg。FV、FVIII和FX的平均血浆半衰期分别为18、43和33小时。除1例因中枢神经系统外科急症接受治疗外，所有患者均接受了有出血风险的择期手术的替代治疗（14例手术，2例阴道分娩）。
16例患者中有13例（81%）的SD血浆治疗疗程被判定为完全有效。在其余3例中，1例FV缺乏且因子水平为43%的患者在大手术后出现轻度出血，1例FXI缺乏且因子水平在20%至41%之间的患者也在大手术后出现轻度出血；1例FV和FVIII联合缺乏且因子水平分别为41%和18%的患者在小手术后出现轻度出血。通过继续或增加SD血浆治疗，出血得到了控制。
这些结果表明，尽管目前与新鲜冷冻血浆相关的血源感染绝对风险相对较小，但在无法获得病毒灭活单因子浓缩物且需要替代治疗的隐性遗传性凝血障碍患者中，应优先选择SD血浆。
