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获得性血友病 A.

ACQUIRED HAEMOPHILIA A.

机构信息

Department of Internal Medicine, Hospital Taiping, Perak, Malaysia.

出版信息

J Ayub Med Coll Abbottabad. 2023 Oct-Dec;35(4):690-692.

Abstract

Acquired haemophilia A (AHA) is a rare disease believed to be caused by spontaneous inhibition of clotting Factor VIII by autoantibodies. This is in contrast to the more common congenital haemophilias which are largely due to an absolute deficiency in coagulation factors. It has a prevalence of approximately one per million per year. However, this figure may be underestimated because there are many undocumented cases due to a lack of recognition. Patients who develop this disease may present with catastrophic bleeding despite having no previous bleeding history. In this study, we report a case of acquired Haemophilia A presenting with spontaneous unprovoked bruising and discuss the approach to diagnosis and how to alert the clinician to suspect this potentially rare but devastating disease.

摘要

获得性血友病 A(AHA)是一种罕见的疾病,据信是由自身抗体自发抑制凝血因子 VIII 引起的。这与更为常见的先天性血友病形成对比,后者主要是由于凝血因子的绝对缺乏。其年发病率约为每百万分之一。然而,由于缺乏认识,可能还有许多未记录在案的病例,因此这个数字可能被低估了。患有这种疾病的患者尽管以前没有出血史,但可能会出现灾难性出血。在本研究中,我们报告了一例表现为自发性无诱因瘀斑的获得性血友病 A 病例,并讨论了诊断方法以及如何提醒临床医生怀疑这种潜在的罕见但具有破坏性的疾病。

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