Viswabandya A, Mathews V, George B, Nair S C, Baidya S, Mammen J J, Chandy M, Srivastava A
Department of Haematology, Christian Medical College, Vellore, Tamil Nadu, India.
Haemophilia. 2008 Jul;14(4):763-7. doi: 10.1111/j.1365-2516.2008.01755.x. Epub 2008 Apr 24.
This report describes our experience with Koate DVI, a factor VIII (FVIII) concentrate containing von Willebrand factor (VWF) for surgery in patients with von Willebrand's disease (VWD). Twenty-one patients underwent 26 procedures, 10 of which were major and 16 were minor. The median age was 27 years (3-55) and the mean weight was 52 kg (16-88). Among the ten patients (type 2-5; type 3-5) who underwent major procedures, the pre-operative dose was 35 IU kg(-1) of FVIII followed by 10-20 IU kg(-1) once daily depending on FVIII:C levels. The mean total dose of FVIII used per procedures was 106 IU kg(-1) (30-190) over a mean duration of 7 days (3-11). In this group, pre-infusion FVIII:C, VWF:Ag and VWF: ristocetin cofactor (RCoF) level that were 19.5% (1-64), 20 U dL(-1) (0-96) and 12% (0-66) increased to 72% (54-198), 131 U dL(-1) (68-206) and 68% (27-108) postinfusion, respectively. Sixteen minor procedures were performed in 11 patients (type 1-3, type 2-6, type 3-2). The preparative dose of FVIII was 10-20 IU kg(-1). The average duration of factor support was 2 days (1-3) for a mean total dose of 23 IU kg(-1) (9-60). The pre-infusion levels of FVIII:C, VWF:Ag and VWF:ristocetin cofactor (RCo) which were 31% (22-64), 25.5 U dL(-1) (0-63) and 21% (0-76), respectively, increased to 76% (27-111), 73 U dL(-1) (30-137) and 45% (2-106) postinfusion. Whereas surgical haemostasis was achieved in all patients, minor postoperative bleeding occurred after one procedure in each group. Both were controlled with additional doses of factor replacement. We conclude that Koate DVI in modest doses provide adequate haemostasis for surgery in patients with VWD.
本报告描述了我们使用Koate DVI（一种含血管性血友病因子（VWF）的凝血因子VIII（FVIII）浓缩剂）治疗血管性血友病（VWD）患者手术的经验。21例患者接受了26次手术，其中10次为大手术，16次为小手术。患者中位年龄为27岁（3 - 55岁），平均体重为52千克（16 - 88千克）。在接受大手术的10例患者（2型 - 5例；3型 - 5例）中，术前FVIII剂量为35 IU kg⁻¹，随后根据FVIII:C水平每天给予10 - 20 IU kg⁻¹。每次手术FVIII的平均总用量为106 IU kg⁻¹（30 - 190），平均持续时间为7天（3 - 11天）。该组患者输注前FVIII:C、VWF:Ag和VWF:瑞斯托霉素辅因子（RCoF）水平分别为19.5%（1 - 64）、20 U dL⁻¹（0 - 96）和12%（0 - 66），输注后分别升至72%（54 - 198）、131 U dL⁻¹（68 - 206）和68%（27 - 108）。11例患者（1型 - 3例，2型 - 6例，3型 - 2例）进行了16次小手术。FVIII的预处理剂量为10 - 20 IU kg⁻¹。因子支持的平均持续时间为2天（1 - 3天），平均总剂量为23 IU kg⁻¹（9 - 60）。输注前FVIII:C、VWF:Ag和VWF:瑞斯托霉素辅因子（RCo）水平分别为31%（22 - 64）、25.5 U dL⁻¹（0 - 63）和21%（0 - 76），输注后分别升至76%（27 - 111）、73 U dL⁻¹（30 - 137）和45%（2 - 106）。虽然所有患者均实现了手术止血，但每组各有1例患者术后出现轻微出血。两者均通过额外剂量的因子替代治疗得到控制。我们得出结论，适度剂量的Koate DVI可为VWD患者手术提供足够的止血效果。
