少年系统性红斑狼疮并发获得性血管性血友病:一例罕见病例,诊断困难

Noor Hayati Sabtu, F. Idris, Eusni Rahayu Tohit, A. Abu Bakar, Wan Aswani Wan Yusof, R. Osman
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引用次数: 0

摘要

在系统性红斑狼疮(SLE)中,血液学异常是常见的,尽管它们是获得性血管性血友病综合征(AVWS)的罕见原因。AVWS是一种罕见的疾病,可导致出血性疾病。我们报告一例AVWS在SLE早期诊断中的应用。入院前一个月,患者有复发性鼻出血史。他以有症状的贫血来医院就诊,并被诊断为严重贫血伴缺铁。住院期间,复发性鼻出血复发,发现活化部分凝血活素时间(aPTT)延长,存在狼疮抗凝剂(LA),血管性血友病因子(VWF)和因子8 (VIII)水平降低。同时,根据系统性狼疮国际合作诊所(SLICC)的标准,他被诊断为SLE。他接受了输血和免疫抑制药物治疗,如类固醇、霉酚酸酯和抗纤溶剂;患者随后止血,临床好转。
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A Teenage Boy with Systemic Lupus Erythematosus Complicated with Acquired von Willebrand Syndrome: A Rare Case and Challenging in Making Diagnosis
In systemic lupus erythematosus (SLE), haematological abnormalities are frequent, although they are an uncommon cause of acquired von Willebrand syndrome (AVWS). AVWS is a rare condition that can cause a bleeding disorder. We presented a case of AVWS in the early diagnosis of SLE. One month before admission, the patient had a history of recurrent epistaxis. He presented to the hospital with symptomatic anaemia and was noted to have severe anaemia with iron deficiency. During hospitalisation, recurrent epistaxis recurred and was found to have prolonged activated partial thromboplastin time (aPTT), presence of lupus anticoagulant (LA), and lower von Willebrand factor (VWF), and factor 8 (VIII) levels. Simultaneously, he was diagnosed with SLE based on Systemic Lupus International Collaborating Clinics (SLICC) criteria. He underwent blood transfusions and was treated with immunosuppressive drugs such as steroids, mycophenolate mofetil, and an anti-fibrinolytic agent; he subsequently stopped bleeding and showed clinical improvement.
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来源期刊
CiteScore
0.50
自引率
0.00%
发文量
28
期刊介绍: The Malaysian Journal of Medicine and Health Sciences (MJMHS) is published by the Faculty of Medicine and Health Sciences, Universiti Putra Malaysia. The main aim of the MJMHS is to be a premier journal on all aspects of medicine and health sciences in Malaysia and internationally. The focus of the MJMHS will be on results of original scientific research and development, emerging issues and policy analyses pertaining to medical, biomedical and clinical sciences.
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