产后获得性血友病 A。

IF 0.9 Q3 MEDICINE, GENERAL & INTERNAL
Khawaja O Omar, William Sebastian, Suzanne Kemper
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引用次数: 0

摘要

获得性血友病 A(AHA)是一种出血性疾病,由针对因子 VIII 的自身抗体异常产生引起。AHA 的根本原因尚不清楚,但可在产后出现。极少数病例报告 AHA 与镰状细胞性状(SCT)等其他血液病同时存在。虽然这种情况很少见,而且两者之间也没有直接的关联,但涉及难治性出血的危急情况会加剧这些疾病的严重性。一名 31 岁的孕妇因镰状细胞性贫血病史而到医院进行剖腹产手术。术后不久,她出现多处顽固性出血。初步化验结果显示,活化部分凝血活酶时间(aPTT)个别升高。进一步检查显示混合研究异常、因子 VIII 活性降低和因子 VIII 抗体存在。患者被诊断为产后 AHA(PAH),并接受了活化因子 VII 和强的松治疗。PAH 是一种不常见的出血性疾病,通常发生在产后一到四个月,表现为出血过多、APTT 升高、混合试验异常、因子 VIII 水平降低和抑制剂水平异常升高。尽管病因不明,但治疗的关键在于止血和消除异常生成的因子 VIII 抑制剂。AHA 与其他血液病的关系尚未阐明。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Postpartum Acquired Hemophilia A.

Acquired hemophilia A (AHA) is a bleeding disorder that occurs from aberrant production of autoantibodies that target factor VIII. The underlying cause of AHA is unclear but can present postpartum. Very few cases have reported instances of AHA coexisting with other hematological disorders, such as sickle cell trait (SCT). Although rare and no direct correlation between the two, critical situations involving intractable bleeding can intensify the severity of these disorders. A 31-year-old pregnant female with a medical history significant for SCT presented to the hospital for a C-section. Shortly after the procedure she experienced intractable bleeding from multiple sites. Initial lab work yielded an isolated increase in activated partial thromboplastin time (aPTT). Further investigation showed abnormal mixing studies, reduced factor VIII activity and the presence of factor VIII antibodies. The patient was diagnosed with postpartum AHA (PAH) and treated with activated Factor VII and prednisone. PAH is an uncommon bleeding disorder that commonly occurs one to four months postpartum and presents as excessive bleeding elevated aPTT, abnormal mixing studies, and reduced factor VIII levels with abnormally high inhibitor levels. Despite an unknown identifiable etiology, treatment hinges upon establishing hemostasis and eradicating the aberrant generated factor VIII inhibitors. The association of AHA and other hematological disorders is not yet elucidated.

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来源期刊
自引率
0.00%
发文量
106
审稿时长
17 weeks
期刊介绍: JCHIMP provides: up-to-date information in the field of Internal Medicine to community hospital medical professionals a platform for clinical faculty, residents, and medical students to publish research relevant to community hospital programs. Manuscripts that explore aspects of medicine at community hospitals welcome, including but not limited to: the best practices of community academic programs community hospital-based research opinion and insight from community hospital leadership and faculty the scholarly work of residents and medical students affiliated with community hospitals.
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