获得性血友病作为系统性红斑狼疮患者的首发表现
Acquired Hemophilia as Initial Presentation in a Patient with Systemic Lupus Erythematosus.
作者信息
Khodamoradi Zohre, Nazarinia Mohammad Ali, Bazdar Somaye
机构信息
Student Research Committee, Shiraz University of Medical Sciences, Shiraz. Iran.
Shiraz Geriatric Research Center, Shiraz University of Medical Sciences, Shiraz. Iran.
出版信息
Curr Rheumatol Rev. 2017;13(3):236-238. doi: 10.2174/1573397113666170519121952.
BACKGROUND
Acquired hemophilia is a rare bleeding disease which might be associated with some autoimmune diseases. Acquired hemophilia may be the result of autoantibodies against factor VIII.
CASE PRESENTATION
In this study, we describe a 55 year old patient who developed hematoma and hematuria due to acquired hemophilia secondary to systemic lupus erythematosus (SLE). Then, she developed arthritis and thrombosis after some evaluations. Laboratory data showed prolonged aPTT, normal PT and platelet, low FVIII, high FVIII inhibitor, ANA, anti-ds-DNA, and anticardiolipin. This patient was treated with corticosteroids, FEIBA and rituximab, so she recovered and was discharged about seven days later.
CONCLUSION
We should consider lupus erythematosus in patients with acquired hemophilia. Some cases of acquired hemophilia may be an SLE manifestation, so we should consider this situation to diagnose the disease properly.
背景
获得性血友病是一种罕见的出血性疾病，可能与某些自身免疫性疾病有关。获得性血友病可能是针对凝血因子 VIII 的自身抗体所致。
病例报告
在本研究中，我们描述了一名 55 岁的患者，因系统性红斑狼疮（SLE）继发获得性血友病而出现血肿和血尿。随后，经过一些评估后，她又出现了关节炎和血栓形成。实验室检查数据显示活化部分凝血活酶时间（aPTT）延长、凝血酶原时间（PT）和血小板正常、凝血因子 VIII（FVIII）水平低、FVIII 抑制剂水平高、抗核抗体（ANA）、抗双链 DNA 和抗心磷脂抗体阳性。该患者接受了皮质类固醇、旁路活化剂（FEIBA）和利妥昔单抗治疗，因此约七天后康复出院。
结论
对于获得性血友病患者，我们应考虑红斑狼疮。一些获得性血友病病例可能是 SLE 的表现，因此我们应考虑这种情况以正确诊断疾病。
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