每日一题_2022.4.18

An older male adult presents to the emergency department (ED) with a chief complaint of paresthesia in his right lower leg and foot. The patient is assessed in the ED triage and a round tumor-like mass is noted on his right anterior thigh. 

The patient reveals that he has had this mass for “a week or so” and that he has been unable to receive regular medical care because he is homeless and uninsured. The patient continues to state that the pain has become so severe that he can no longer move his leg. He denies any history of injury or trauma to the area and denies the use of medically prescribed or recreational use drugs. He shares, “I take a little aspirin here and there for the pain.” 

Diagnostic imaging and serum laboratory tests are immediately ordered, and the healthcare team subsequently discovers the patient has a history of frequent visits to this ED for similar complaints. Review of his electronic health record reveals he has a history of hemophilia A. 

The team confirms the diagnosis with the patient who responds that he had forgotten to mention it and does not receive regular factor VIII replacement therapy. Further assessment, examination, and interventions – including an open excisional biopsy and subsequent amputation – confirm the diagnosis of osseous hemophilic pseudotumor. 

Which perioperative implementation is most important to include?

A. Administration of IV fresh frozen plasma (FFP) 

B. Confirm the client has not taken aspirin in the past 36 hours 

C. Continuous IV infusion of coagulation factor VIlI

D. Prepare the patient for 4 units of packed red blood cells (PRBCs) 

答案解析：

围手术期最重要的实施是为患者提供持续输注凝血因子Ⅷ。患者有严重出血的风险，并且如假瘤所示，出现不受控制的慢性出血。预防重度出血是甲型血友病患者最重要的管理干预，因此建议重度甲型血友病患者（因子活性 < 1%）定期预防性IV给予凝血因子VIII。目前在美国有多个正在进行的用于甲型血友病管理的基因治疗临床试验。虽然这些研究的结果非常有前景，但在基因治疗能够成为甲型血友病的一线标准治疗之前，仍有关键性的研究需要完成。

虽然FFP和PRBC可能对失血和/或与无法凝血相关的失血患者有帮助，但该患者的出血性疾病与缺乏凝血因子VIII（而非低循环血容量）特异性相关。此外，重要的是要注意患者的阿司匹林使用史，因为这可能增加出血风险，但对于正在接受手术的甲型血友病患者，确保阿司匹林停药不是最重要的实施措施。

正确答案：C