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一名患有先前未诊断出的结缔组织病的西班牙裔孕妇出现严重血尿,诊断为获得性因子VIII缺乏症。

Acquired Factor VIII Deficiency Presenting as Gross Hematuria in a Hispanic, Pregnant Patient with Previously Undiagnosed Connective Tissue Disease.

作者信息

Loftis Christine, Dulgheru Emilia C, White Rosa

机构信息

University of Texas at Rio Grande Valley, Internal Medicine Department- Doctor Hospital at Renaissance, 5423 S McColl Rd, Edinburg, TX 78539, USA.

Rheumatology Institute-Doctor Hospital at Renaissance, Edinburg, TX 78539, USA.

出版信息

Case Rep Rheumatol. 2021 Nov 17;2021:3666270. doi: 10.1155/2021/3666270. eCollection 2021.

Abstract

Acquired factor VIII deficiency is a bleeding disorder caused by the presence of autoantibodies against clotting factor VIII. We report a case of a 24-year-old pregnant woman who presented with gross hematuria secondary to acquired factor VIII deficiency in the presence of a previously undiagnosed connective tissue disease. This article includes a literature review of pregnancy-related cases of acquired factor VIII deficiency. We also reviewed various therapeutic approaches for the management of the acquired factor inhibitor which include achieving hemostasis and elimination of the inhibitor via immunosuppressive agents. This case report describes the rare presentation of acquired factor VIII deficiency related to pregnancy and highlights the importance of considering a factor VIII inhibitor in the differential diagnosis of patients who present with bleeding and prolonged PTT during the peripartum and postpartum periods.

摘要

获得性因子VIII缺乏症是一种由针对凝血因子VIII的自身抗体引起的出血性疾病。我们报告一例24岁孕妇,在患有先前未诊断出的结缔组织病的情况下,继发于获得性因子VIII缺乏症而出现肉眼血尿。本文包括对与妊娠相关的获得性因子VIII缺乏症病例的文献综述。我们还回顾了治疗获得性因子抑制剂的各种方法,包括通过免疫抑制剂实现止血和消除抑制剂。本病例报告描述了与妊娠相关的获得性因子VIII缺乏症的罕见表现,并强调了在围产期和产后出现出血和PTT延长的患者的鉴别诊断中考虑因子VIII抑制剂的重要性。

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