Li Yang, Lyu Ming' en, Xue Feng, Liu Wenjie, Hao Yating, Guan Yue, Sun Boyang, Lyu Cuicui, Gu Xueping, Fu Rongfeng, Huang Yueting, Liu Wei, Chen Yunfei, Liu Xiaofan, Zhang Lei, Yang Renchi
Institute of Hematology & Blood Diseases Hospital, CAMS & PUMC, Tianjin 300020, China.
Zhonghua Xue Ye Xue Za Zhi. 2016 Feb;37(2):130-3. doi: 10.3760/cma.j.issn.0253-2727.2016.02.009.
To deepen the understanding of clinical manifestations and treatment of patients with positive lupus anticoagulant (LAC).
The clinical data of 2 patients were analyzed and related literature were reviewed.
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 1:1000 (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 1:1000 (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 II:C was below normal.
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.
加深对狼疮抗凝物(LAC)阳性患者临床表现及治疗的认识。
分析2例患者的临床资料并复习相关文献。
病例1,31岁女性,诊断为狼疮抗凝物阳性,继发于未分化结缔组织病,表现为月经过多和血小板减少。抗核抗体(ANA)1:1000阳性(均质型),抗双链DNA阳性,dRVVT LA1/LA2为3.4。经糖皮质激素和白芍总苷治疗后凝血功能改善。病例2,59岁女性,表现为牙龈出血、血尿,F II:C水平为13%。dRVVT LA1/LA2为2.0。抗核抗体(ANA)1:1000阳性(胞浆颗粒型),抗双链DNA阳性。患者诊断为低凝血酶原血症-狼疮抗凝物综合征(LAHS)及获得性凝血因子缺乏。经甲泼尼龙40mg/天及环磷酰胺治疗后出血症状缓解,但F II:C水平仍低于正常。
LAC阳性患者症状多样。诊断依靠病史及实验室检查。目前尚无标准化治疗。对于LAHS病例,应全面排查任何已知病因及相关疾病。