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获得性血友病A:一种潜在致命的出血性疾病。

Acquired Hemophilia A: A Potentially Fatal Bleeding Disorder.

作者信息

Singh Navdeep, Singh Lubana Sandeep, Dabrowski Lech

机构信息

Hospice and Palliative Care Medicine, North Shore Long Island Jewish Hospital, Manhasset, USA.

Hematology and Medical Oncology, State University of New York Downstate Medical Center, Brooklyn, USA.

出版信息

Cureus. 2020 Jun 21;12(6):e8744. doi: 10.7759/cureus.8744.

Abstract

Acquired hemophilia A (AHA) is a rare autoimmune hematological disorder that has an incidence of about 1.5 cases per million people per year. It occurs in the elderly with the median age of 75 years, and most of the cases are idiopathic. It occurs due to the development of factor VIII inhibitor, which is an autoantibody against factor VIII leading to potentially life-threatening bleeding episodes. The diagnosis of AHA is often delayed and challenging. We report a case of an 86-year-old male who initially presented with signs and symptoms of a stroke. He was found to have oral mucosal bleeding and swelling of the floor of the mouth. He later developed epistaxis, hematuria, and melena. He had an isolated elevation of activated partial thromboplastin time (APTT) with very high levels of factor VIII inhibitor (1152 Bethesda units) and very low levels of Factor VIII (<1%). He was managed with supportive transfusion, bypass agents, and immunosuppressive therapy. AHA is a rare autoimmune bleeding disorder and is more commonly seen in the elderly population. Bleeding in AHA is usually sudden and sometimes life-threatening. Hence early hemostasis with bypassing agents and treatment with immunosuppressive agents should be initiated. Due to the rarity of the disorder, it is crucial to report AHA cases to create awareness and increase the index of suspicion of the clinicians for early diagnosis and treatment to prevent morbidity and mortality.

摘要

获得性血友病A(AHA)是一种罕见的自身免疫性血液系统疾病,年发病率约为每百万人1.5例。该病好发于老年人,中位年龄为75岁,大多数病例为特发性。它是由于VIII因子抑制物的产生所致,VIII因子抑制物是一种针对VIII因子的自身抗体,可导致危及生命的出血发作。AHA的诊断常常延迟且具有挑战性。我们报告一例86岁男性患者,最初表现为中风的症状和体征。他被发现有口腔黏膜出血和口底肿胀。随后出现鼻出血、血尿和黑便。他的活化部分凝血活酶时间(APTT)单独升高,VIII因子抑制物水平非常高(1152贝塞斯达单位),而VIII因子水平非常低(<1%)。他接受了支持性输血、旁路制剂和免疫抑制治疗。AHA是一种罕见的自身免疫性出血性疾病,在老年人群中更为常见。AHA的出血通常突然发生,有时会危及生命。因此,应尽早使用旁路制剂进行止血,并开始使用免疫抑制剂治疗。由于该疾病罕见,报告AHA病例对于提高认识和增加临床医生的怀疑指数以实现早期诊断和治疗从而预防发病和死亡至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3a52/7377015/0676040da3b9/cureus-0012-00000008744-i01.jpg

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