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Acquired Factor VIII Deficiency Presenting as Gross Hematuria in a Hispanic, Pregnant Patient with Previously Undiagnosed Connective Tissue Disease.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。
Case Rep Rheumatol. 2021 Nov 17;2021:3666270. doi: 10.1155/2021/3666270. eCollection 2021.
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本文引用的文献

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Postpartum-Acquired Hemophilia A Presenting as Hemoperitoneum: A Case Report.以腹腔积血为表现的产后获得性甲型血友病:一例报告
Cureus. 2020 Dec 1;12(12):e11817. doi: 10.7759/cureus.11817.
2
2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus.2019 年欧洲抗风湿病联盟/美国风湿病学会系统性红斑狼疮分类标准。
Ann Rheum Dis. 2019 Sep;78(9):1151-1159. doi: 10.1136/annrheumdis-2018-214819. Epub 2019 Aug 5.
3
Pregnancy-related acquired hemophilia A initially manifesting as pleural hemorrhage: A case report.妊娠相关获得性甲型血友病最初表现为胸腔出血:一例报告
Medicine (Baltimore). 2019 Jan;98(3):e14119. doi: 10.1097/MD.0000000000014119.
4
Acquired factor VIII deficiency: two case reports and a review of literature.获得性因子VIII缺乏症:两例病例报告及文献综述
Exp Hematol Oncol. 2017 Mar 24;6:8. doi: 10.1186/s40164-017-0068-3. eCollection 2017.
5
An extra X does not prevent acquired hemophilia - Pregnancy-associated acquired hemophilia A.额外的一条X染色体并不妨碍获得性血友病——妊娠相关获得性甲型血友病。
Thromb Res. 2017 Mar;151 Suppl 1:S82-S85. doi: 10.1016/S0049-3848(17)30074-9.
6
Pregnancy exacerbates complications of acquired hemophilia in a patient with systemic lupus erythematosus.
Postepy Dermatol Alergol. 2015 Jun;32(3):235-8. doi: 10.5114/pdia.2014.40964. Epub 2015 Jun 15.
7
Pregnancy Associated with Systemic Lupus Erythematosus: Immune Tolerance in Pregnancy and Its Deficiency in Systemic Lupus Erythematosus--An Immunological Dilemma.妊娠与系统性红斑狼疮:孕期的免疫耐受及其在系统性红斑狼疮中的缺陷——一个免疫学困境
J Immunol Res. 2015;2015:241547. doi: 10.1155/2015/241547. Epub 2015 May 18.
8
Second case of postpartum acquired hemophilia A in a Korean female.
Blood Res. 2014 Sep;49(3):205-7. doi: 10.5045/br.2014.49.3.205. Epub 2014 Sep 25.
9
Nonlinear pharmacokinetics of factor VIII and its phosphatidylinositol lipidic complex in hemophilia A mice.血友病 A 小鼠体内因子 VIII 及其磷脂酰肌醇脂质复合物的非线性药代动力学。
Biopharm Drug Dispos. 2014 Apr;35(3):154-63. doi: 10.1002/bdd.1880. Epub 2014 Jan 2.
10
Postpartum acquired hemophilia: a rare cause of postpartum hemorrhage.
Case Rep Hematol. 2013;2013:735715. doi: 10.1155/2013/735715. Epub 2013 Feb 28.

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.

DOI:10.1155/2021/3666270
PMID:34840848
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8612772/
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。