首页|罕见凝血因子缺乏症患者围术期输血策略——附1例凝血因子Ⅺ缺乏症伴膝关节血管瘤的病例报告及文献复习

罕见凝血因子缺乏症患者围术期输血策略——附1例凝血因子Ⅺ缺乏症伴膝关节血管瘤的病例报告及文献复习

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目的 通过对1例凝血因子Ⅺ缺乏症伴膝关节血管瘤患者的围术期凝血管理,探讨遗传性凝血因子缺乏症患者围术期的输血策略。方法 采用凝血因子活性检查和基因检测,确诊患者为凝血因子Ⅺ缺乏症,通过围术期出血风险评估,血栓弹力图以及凝血指标检测来适时监测患者的凝血状态。术前输注新鲜冰冻血浆(FFP)400 mL,术后d1输注FFP 400 mL,此后3 d每日再输注200 mL。结果 患者APTT术前为97。1 s,术后为36。9 s,术中无明显出血,术后伤口愈合情况良好。结论 在缺乏凝血因子制剂的情况下,凝血因子缺乏症患者在围术期可以通过积极输注FFP等血液制品来显著改善患者的凝血功能,降低术中出血的风险。
Perioperative transfusion strategies for patients with rare coagulation factor deficiency:a case report of coagulation factor antecedent deficiency with hemangioma of knee joint and literature review
Objective To discuss the perioperative blood transfusion strategy of patients with hereditary coagulation fac-tor deficiency through the perioperative blood coagulation management of a patient with coagulation factor Ⅺ deficiency and hemangioma of knee joint.Methods Coagulation factor activity test and gene test were used to confirm the patients with co-agulation factor Ⅺ antecedent deficiency.Perioperative bleeding risk assessment,thromboela-stogram(TEG)and coagula-tion factors detection were performed to detect the coagulation status of the patients.400 mL fresh frozen plasma was trans-fused before surgery and first day after surgery,200 mL daily for 3 days thereafter.Results The level of APTT was con-trolled from 97.1 s before operation to 36.9 s after operation.There was no obvious bleeding during operation,and the wound healed well after operation.Conclusion In the situation of lack of commercial coagulation factors,transfusion of blood products such as fresh frozen plasma can significantly improve the coagulation function and reduce the risk of intraoperative bleeding in patients with hereditary coagulation factor deficiency during perioperative period.

rare coagulation factor deficiencyblood transfusionperioperative

索正新、王莉、牛迎迎、黄春妍

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四川大学华西医院输血科,四川成都 610041

四川大学华西天府医院输血科,四川成都 610041

罕见凝血因子缺乏症 输血 围术期

2024

中国输血杂志
中国输血协会 中国医学科学院输血研究所

中国输血杂志

CSTPCD
影响因子:1.279
ISSN:1004-549X
年,卷(期):2024.37(10)