Perioperative management of a patient with hemophilia a and high-titer inhibitors and literature review
10.3760/cma.j.cn121090-20250113-00026
- VernacularTitle:1例血友病A合并高滴度抑制物患者胸腔镜肺叶切除术的围手术期止血管理及文献复习
- Author:
Zhijun MENG
1
;
Jie XIANG
;
Guanqun XU
;
Yu LIU
;
Qiulan DING
;
Jing DAI
;
Wenman WU
;
Xuefeng WANG
Author Information
1. 山西省人民医院检验科，山西 030012
- Publication Type:Journal Article
- From:
Chinese Journal of Hematology
2025;46(8):766-768
- CountryChina
- Language:Chinese
-
Abstract:
A 64-year-old male patient with hemophilia A was scheduled for the surgical removal of a pulmonary mass. Preoperative evaluation revealed that the coagulation factor Ⅷ (FⅧ) activity was 0.5%, with an F Ⅷ inhibitor level of 32 BU/ml; the R value could not be detected on the thromboelastogram. Thoracoscopic lobectomy was successfully completed. On the day of the operation and the first day after the operation, 6 mg of recombinant activated coagulation factor Ⅶ (rFⅦa) was intravenously administered every 6 h. On postoperative day 1, the patient’s blood pressure dropped and the HGB gradually declined from 102 g/L to 65 g/L. Chest X-ray revealed a large amount of pleural effusion on the left side, and urgent thoracoscopic thoracic exploration was performed. A total of 3200 mL fresh blood was cleared, and a thoracic drainage tube was placed. On postoperative day 2, the rFⅦa dose was increased to 6 mg, which was intravenously administered every 4 h, and concentrated red cells were intermittently infused to correct anemia. Four days later, due to the inability to obtain rFⅦa, PCC (50 IU/kg every 8 hours) was administered. Additionally, treatment with methylprednisolone (40 mg/d) and cyclophosphamide (200 mg, every 2 weeks) was initiated to remove FⅧ inhibitors. The thoracic drainage tube was removed on postoperative day 9, and the patient was successfully discharged 3 weeks later.
