狼疮抗凝物二例报告并文献复习

Lupus anticoagulant： two cases report and literature review

目的 加深对狼疮抗凝物所致凝血异常的认识.方法 回顾性分析2例狼疮抗凝物患者的临床特征、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 例1,女,31岁,因月经量多4年入院;PT、APTT延长,血浆蝰蛇毒时间（dRVVT）筛选试验（LA1） 195.5 s,确诊试验（LA2） 57.4 s,LA 1/LA2=3.4,抗心磷脂抗体IgG阳性,凝血因子Ⅱ、Ⅷ、Ⅸ、Ⅺ、Ⅻ活性下降（经稀释后均恢复正常）,抗核抗体1：1 000阳性（均质型核仁型）,抗双链DNA阳性;诊断：凝血功能异常（狼疮抗凝物）,结缔组织病;予以糖皮质激素、白芍总苷及对症治疗,凝血功能恢复.例2,女,59岁,因齿龈出血、血尿1个月入院;dRVVT试验LA1/LA2=2.0,凝血因子Ⅱ活性13％,凝血因子Ⅶ、Ⅷ、Ⅸ、Ⅺ、Ⅻ活性均下降（稀释后凝血因子Ⅱ活性8％,其他凝血因子活性恢复正常）;抗核抗体1：1 000阳性（胞质颗粒型）,ENA抗体谱均阴性;诊断：低凝血酶原血症-狼疮抗凝物综合征;应用甲泼尼龙40 mg/d联合小剂量环磷酰胺并间断输注凝血酶原复合物治疗,出血症状改善,凝血因子Ⅱ活性仍低于正常,仍有关节血肿反复发作.结论 狼疮抗凝物所致凝血异常患者的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.

Objective To deepen the understanding of clinical manifestations and treatment of patients with positive lupus anticoagulant （LAC）.Methods The clinical data of 2 patients were analyzed and related literature were reviewed.Results Case 1,a 31-year-old female,diagnosed as lupus anticoagulant positive,secondary to undifferentiated connective tissue disease,was presented with menorrhagia and thrombocytopenia.Anti-nuclear antibody （ANA） was positive 1：1 000 （homogeneous type） with anti-double stranded DNA positive,and dRVVT LA1/LA2 was 3.4.Coagulation function was alleviated after treatment with glucocorticoid and total glucosides of paeony.Case 2,a 59-year-old female was presented with gingival bleeding,hematuria with the level of F Ⅱ：C 13%.dRVVT LA1/LA2 was 2.0.Anti-nuclear antibody （ANA） was positive 1：1 000 （type of cytoplasmic granule）,anti-double stranded DNA was positive.The patient was diagnosed as hypoprothrombinemia-lupus anticoagulant syndrome （LAHS） and acquired coagulation factor deficiency.The signs of hemorrhage were alleviated after treatment with methylprednisolone 40 mg/day and cyclophosphamide,while the level of F IⅡ：C was below normal.Conclusion Symptoms of patients with positive LAC are variable.The diagnosis relies on history of disease and laboratory test.Currently,there is no standardized treatment.Cases of LAHS should be thoroughly investigated for any known causes and related disorder.