# Perioperative management of a patient with hemophilia A and high-titer inhibitors and literature review

## TL;DR

A 64-year-old hemophilia A patient with high FⅧ inhibitors successfully underwent surgery with careful perioperative management and treatment adjustments.

## Contribution

Presents a case study on managing hemophilia A with high-titer inhibitors during surgery, emphasizing treatment strategies and outcomes.

## Key findings

- rFⅦa was effective in managing bleeding during and after the initial surgery.
- Postoperative complications required increased rFⅦa dosage and additional interventions like blood transfusions.
- Switching to prothrombin complex concentrate and immunosuppressive therapy helped manage the patient's condition after rFⅦa shortage.

## Abstract

1例64岁男性血友病A患者，因“肺占位”拟行手术治疗。术前发现凝血因子Ⅷ（FⅧ）活性0.5％、FⅧ抑制物32 BU/ml，血栓弹力图R值无法检测。顺利完成胸腔镜肺叶切除术，手术当天及术后第1天予以活化凝血因子Ⅶ（rFⅦa）6 mg每6 h 1次静脉滴注。术后第1天发现血压下降、HGB由102 g/L逐渐降至65 g/L，X线胸片显示左侧大量胸腔积液；急行胸腔镜下胸腔探查，清理新鲜积血3 200 ml并放置胸腔引流管。第2次术后，rFⅦa加量至6 mg每4 h 1次静脉滴注，间断输注去白红细胞纠正贫血。4 d后因rFⅦa不能获得，改用凝血酶原复合物50 IU/kg每8 h 1次，同时予以清除FⅧ抑制物治疗（甲泼尼龙40 mg/d、环磷酰胺200 mg每周2次）。术后第9天拔除胸腔引流管，术后3周痊愈出院。