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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

Pregnancy-related acquired hemophilia A initially manifesting as pleural hemorrhage: A case report.

作者信息

Qian Lili, Ge Hangping, Hu Pingping, Zhu Ni, Chen Junfa, Shen Jianping, Zhang Yu

机构信息

Department of Laboratory.

Department of Hematology, First Affiliated Hospital of Zhejiang, Chinese Medical University, Hangzhou, China.

出版信息

Medicine (Baltimore). 2019 Jan;98(3):e14119. doi: 10.1097/MD.0000000000014119.

Abstract

RATIONALE

Acquired hemophilia A is a rare hemorrhagic disease in which the body produces specific antibodies that attack factor VIII, resulting in bleeding that is mainly mucocutaneous and associated with soft tissue and the gastrointestinal system. Approximately 50% of this disease derives from basic diseases, such as autoimmune diseases, cancer, and pregnancy.

PATIENT CONCERNS

We report a 35-year-old postpartum female with acquired hemophilia A who initially presented with pleural hemorrhage.

DIAGNOSES

In this patient activated prothrombin time (PT) and activated partial thromboplastin time (APTT) were found, and the factor VIII activity was 12.6%, furthermore Bethesda assay showed a FVIII antibody titer of 7.4 Bethesda units (BUs).

INTERVENTIONS

The treatment requires a 2-pronged approach: treatment of the bleeding and elimination of the inhibitor.

OUTCOMES

After hemostatic agents were used and inhibitors were eradicated, the patient achieved complete remission without relapse.

LESSONS

It is essential to recognize the development of disease earlier in pregnant woman.

摘要

理论依据

获得性血友病A是一种罕见的出血性疾病,机体产生攻击凝血因子VIII的特异性抗体,导致主要为黏膜皮肤出血,并与软组织和胃肠道系统相关。约50%的该疾病源于基础疾病,如自身免疫性疾病、癌症和妊娠。

患者情况

我们报告一名35岁产后获得性血友病A女性患者,最初表现为胸腔出血。

诊断

该患者活化凝血酶原时间(PT)和活化部分凝血活酶时间(APTT)延长,凝血因子VIII活性为12.6%,此外,贝塞斯达检测显示FVIII抗体滴度为7.4贝塞斯达单位(BU)。

干预措施

治疗需要采取双管齐下的方法:治疗出血和消除抑制剂。

结果

使用止血剂并根除抑制剂后,患者完全缓解且未复发。

经验教训

对于孕妇,尽早识别疾病的发展至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9664/6370013/aaa3349b61d5/medi-98-e14119-g001.jpg

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