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合并危及生命的内脏器官出血及与获得性血友病A相关的产后出血。

Combined life-threatening internal organ bleeding and postpartum hemorrhage associated with acquired hemophilia A.

作者信息

Kanitthamniyom Chanakarn, Siladech Pharit, Polpichai Natchaya, McCullough Maireigh, Saowapa Sakditad

机构信息

Division of Hematology Sirindhorn Hospital Bangkok Thailand.

Faculty of Medicine Ramathibodi Hospital Bangkok Thailand.

出版信息

Clin Case Rep. 2024 Jan 2;12(1):e8399. doi: 10.1002/ccr3.8399. eCollection 2024 Jan.

Abstract

KEY CLINICAL MESSAGE

Acquired hemophilia A (AHA) can present as life-threatening bleeding during the postpartum period. Prompt treatment allows patients with AHA to achieve complete remission and have normal subsequent pregnancies.

ABSTRACT

Acquired hemophilia A (AHA) is a rare bleeding disorder caused by the production of autoantibodies against factor VIII (FVIII). AHA can present with severe bleeding, especially in postpartum patient. We report a 38-year-old woman who presented in an emergency department with severe postpartum hemorrhage 2 weeks after cesarean section. Her investigation showed an isolated prolongation of partial thromboplastin time (PTT), low factor VIII assay and a factor VIII inhibitor test, resulting in abnormal Bethesda units which consistent with AHA. This case report highlights the importance of early diagnosis and treatment of AHA. With timely and appropriate management, most patients can achieve a good outcome.

摘要

关键临床信息

获得性血友病A(AHA)可在产后表现为危及生命的出血。及时治疗可使AHA患者实现完全缓解,并在随后的妊娠中正常妊娠。

摘要

获得性血友病A(AHA)是一种罕见的出血性疾病,由针对凝血因子VIII(FVIII)的自身抗体产生所致。AHA可表现为严重出血,尤其是产后患者。我们报告一名38岁女性,剖宫产术后2周因严重产后出血就诊于急诊科。她的检查显示部分凝血活酶时间(PTT)单独延长、FVIII检测值低以及FVIII抑制物试验,导致贝塞斯达单位异常,符合AHA。本病例报告强调了AHA早期诊断和治疗的重要性。通过及时、恰当的管理,大多数患者可取得良好预后。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a03b/10761610/f34c7845c9f3/CCR3-12-e8399-g001.jpg

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