获得性凝血因子V抑制物患者三例报告并文献复习

Acquired factor V inhibitor： 3 cases report and literature review

目的提高对获得性凝血因子V抑制物所致凝血障碍的认识。方法报告3例获得性凝血因子V抑制物阳性患者的临床特征、实验室检查指标、诊治经过及转归，并进行相关文献复习和讨论。结果3例患者年龄均在50岁以上，无遗传性凝血因子缺乏病史及家族史，亦无潜在相关疾病。轻者表现为自发性皮肤黏膜出血或肉眼血尿、黑便、重者表现为颅内出血。实验室检查均有凝血酶原时间（PT）、活化部分凝血活酶时间（APTT）显著延长、凝血因子V活性（FV：C）降低、抑制物定性实验或Bethesda法定量阳性。经过糖皮质激素及免疫抑制治疗，2例患者临床症状明显改善，原有出血停止，无新发出血表现，但凝血指标、FV：C恢复缓慢；另1例死于颅内出血。结论获得性凝血因子V抑制物所致凝血障碍是一种少见疾病，临床表现具有异质性，出血症状轻重不一；小剂量糖皮质激素及免疫抑制剂治疗有效；预后与患者基础疾病相关。

Objective To better understand the acquired factor V （ F V ） inhibitors. Methods The clinical features, laboratory manifestations, treatment options and prognosis of 3 cases were reported and related literature were reviewed. Results All the 3 patients were older than 50 years without family history and related disease. Their clinical manifestations included spontaneously mucous bleeding, hematuria, epi- staxis and eneephalie bleeding. Laboratory test showed prolonged prothrombin time （PT） and activated partial thromboplastin time （AFIT）. The F V levels decreased and the presence of F V inhibitor was confirmed by Bethesda method. All patients were treated with glucocorticoid and immunosuppressive agents. The haemor- rhages of two patients stopped but their coagulation test and F V level recovered slowly. One patient died from encephalie bleeding. Conclusions Acquired F V inhibitor is a rare coagulation disorder with variable clinical symptoms. Immunosuppressive agents are effective to eliminate the inhibitors. The prognosis of acquired F V inhibitors seemed to be strictly related to the basic disease.