获得性凝血因子Ⅹ缺乏症三例报告并文献复习

Acquired coagulation factor Ⅹ deficiency： three cases report and literature review

目的 加深对获得性凝血因子Ⅹ缺乏症的认识.方法 对3例获得性凝血因子Ⅹ缺乏症患者的临床资料进行分析,并复习相关文献.结果 例1,男,57岁,诊断为多发性骨髓瘤轻链型、继发性淀粉样变、获得性凝血因子Ⅹ缺乏症,表现为自发性皮肤黏膜出血,凝血因子Ⅹ活性（FⅩ∶C）1.8％,予以MP（马法兰＋曲安西龙）方案联合沙利度胺及对症治疗,FⅩ∶C未见升高,因原发病进展死亡.例2,男,41岁,以颅内出血入院,FⅩ∶C 26.8％,予以补充叶酸、维生素B12、维生素K,并输注红细胞、血小板及新鲜冰冻血浆治疗,颅内出血好转.例3,女,63岁,因反复发作四肢关节出血4个月入院,FⅩ∶C 6.1％,给予凝血酶原复合物、甲泼尼龙、硫唑嘌呤、利妥昔单抗治疗,FⅩ∶C未见明显升高,关节腔出血仍反复发作.结论 获得性凝血因子Ⅹ缺乏症临床表现具有异质性,诊断依赖病史和实验室检查,治疗包括控制出血和治疗原发病,预后与患者基础疾病相关.

Objective To deepen the understanding of acquired coagulation factor Ⅹ （F Ⅹ） deficiency.Methods The clinical data of 3 patients were analyzed and related literature were reviewed.Results Case 1,a 57-year-old male,secondary to multiple myeloma and amyloidosis,was presented with spontaneous mucous hemorrhage with the level of F Ⅹ ∶ C 1.8％,which kept unchanged after chemotherapy with melphalan,glucocorticoid,and thalidomide,and died of primary disease progression.Case 2,a 41-year-old male with psoriasis,was presented with cerebral and retinal hemorrhage with the level of F Ⅹ ∶ C 26.8％.The signs of hemorrhage were alleviated after the supplement of folic acid,vitamin B12,and vitamin K,and transfusion with red blood cells,platelets,and fresh frozen plasma.Case 3,a 63-year-old female,associated with high level of lupus anticoagulant,was presented with repeated ecchymosis and haemarthrosis with the level of F Ⅹ ∶C 6.1％,which was refractory to prothrombin complex concentrate,methyprednisolone,azathioprine,and rituximab.Conclusions Acquired F Ⅹ deficiency is a rare disorder with variable symptoms.The diagnosis relies on history of disease and laboratory test.Currently,there is no standardized treatment.The prognosis of acquired F Ⅹ deficiency is mainly related to the underlying disease.