Department of Pediatrics, Nara Medical University, Kashihara, Nara, Japan.
Clin Appl Thromb Hemost. 2012 Jan-Feb;18(1):66-71. doi: 10.1177/1076029611412364. Epub 2011 Jul 6.
We have utilized high-dose factor VIII (FVIII) concentrates in 4 hemophilia A patients with inhibitors prior to surgery for the insertion of a central venous access device. In total, 3 patients out of 4 had high responding inhibitors. Dosing algorithms for this type of therapy have not been previously validated and established. We devised an effective formula to calculate the initial dose required to neutralize the inhibitors, although some of the patients demonstrated a lower recovery of FVIII than expected. An anamnestic inhibitor response was evident in 3 cases, but overall our strategy provided a reliable hemostatic effect for at least 4 days after surgery. In addition, our protocol appeared to be more cost-effective than FVIII bypass therapy. The financial saving in 1 case for the initial 3 days was estimated to be approximately US$49 122. Our results demonstrated that high-dose FVIII therapy provided clinically effective and economically viable results even in high responders.
我们在 4 名接受手术置入中心静脉通路装置的有抑制剂的甲型血友病患者中使用了高剂量的凝血因子 VIII(FVIII)浓缩物。在这 4 名患者中,有 3 名患者的抑制剂呈高反应性。这种治疗类型的剂量算法以前没有经过验证和确立。我们设计了一种有效的公式来计算中和抑制剂所需的初始剂量,尽管有些患者的 FVIII 恢复情况低于预期。3 例患者出现了既往抑制剂反应,但总的来说,我们的策略至少在手术后 4 天内提供了可靠的止血效果。此外,我们的方案似乎比 FVIII 旁路治疗更具成本效益。在 1 例患者中,最初 3 天的节省费用估计约为 49122 美元。我们的结果表明,即使是高反应者,高剂量 FVIII 治疗也能提供临床有效且经济可行的结果。