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[狼疮抗凝物:两例报告及文献综述]

[Lupus anticoagulant: two cases report and literature review].

作者信息

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

机构信息

Institute of Hematology & Blood Diseases Hospital, CAMS & PUMC, Tianjin 300020, China.

出版信息

Zhonghua Xue Ye Xue Za Zhi. 2016 Feb;37(2):130-3. doi: 10.3760/cma.j.issn.0253-2727.2016.02.009.

Abstract

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 1:1000 (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.

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.

摘要

目的

加深对狼疮抗凝物(LAC)阳性患者临床表现及治疗的认识。

方法

分析2例患者的临床资料并复习相关文献。

结果

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。

结论

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。

相似文献

1
[Lupus anticoagulant: two cases report and literature review].[狼疮抗凝物:两例报告及文献综述]
Zhonghua Xue Ye Xue Za Zhi. 2016 Feb;37(2):130-3. doi: 10.3760/cma.j.issn.0253-2727.2016.02.009.
4
The (reverse) paradox of lupus anticoagulant: A case report.狼疮抗凝物的(反向)悖论:一例报告。
Lupus. 2024 Oct;33(12):1383-1388. doi: 10.1177/09612033241282058. Epub 2024 Sep 7.

本文引用的文献

2
The paradox of the lupus anticoagulant: history and perspectives.狼疮抗凝物的悖论:历史与展望
Semin Thromb Hemost. 2014 Nov;40(8):860-5. doi: 10.1055/s-0034-1395158. Epub 2014 Nov 11.
7
Bleeding in the antiphospholipid syndrome.抗磷脂综合征中的出血
Hematology. 2012 Apr;17 Suppl 1:S153-5. doi: 10.1179/102453312X13336169156654.
8
Guidelines on the investigation and management of antiphospholipid syndrome.抗磷脂综合征的调查与管理指南
Br J Haematol. 2012 Apr;157(1):47-58. doi: 10.1111/j.1365-2141.2012.09037.x. Epub 2012 Feb 8.

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