Acquired (autoimmune) hemophilia: demographics, outcomes, and readmissions

Blood Vessels, Thrombosis & Hemostasis Pub Date : 2025-02-16 DOI:10.1016/j.bvth.2025.100062

Aditi Sharma , Nikhil Vojjala , Vijendra Singh

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Abstract

Acquired hemophilia (AHA) is a rare bleeding disorder characterized by autoantibodies against coagulation factors. It predominantly affects older adults and is associated with autoimmune disorders or malignancies. Achieving hemostasis and inhibiting autoantibody production through immunosuppression are the mainstays of treatment.We conducted a retrospective cohort study using the Nationwide Readmissions Database from 2016 to 2019 to perform a descriptive analysis of AHA, including the estimation of the 30-day readmission rate and primary diagnoses at readmission.Of 1450 admissions for AHA, 803 (55.4%) were male, and the median age at admission was 73 years. Approximately 21% had an underlying solid malignancy, 3.9% had hematologic malignancy, and 13.5% had autoimmune disease. Acute myocardial infarction occurred in 9.5%, disseminated intravascular coagulation in 1.2%, intracranial hemorrhage in 1.5%, ischemic stroke in 2.5%, and venous thromboembolism in 4.4%. Bleeding occurred in 30.2% of admissions, with 27% requiring blood transfusion. The median length of hospital stay was 7 days, and there were 101 deaths during the index admission, resulting in a 7% inpatient mortality rate. Mortality was higher in the older age group at 8% compared with 3% in those aged <65 years (P = .03). The 30-day readmission rate was 27%, with a 10.8% mortality rate during readmission. Infections followed by bleeding were the most common causes for readmission. High rates of bleeding and thrombotic complications are seen in AHA, with bleeding and infection being the primary reasons for the elevated 30-day readmission rate, indicating significant treatment-related toxicity and disease relapse.

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获得性（自身免疫性）血友病：人口统计学、结果和再入院

摘要获得性血友病（AHA）是一种罕见的以自身抗凝血因子抗体为特征的出血性疾病。它主要影响老年人，并与自身免疫性疾病或恶性肿瘤有关。通过免疫抑制实现止血和抑制自身抗体的产生是治疗的主要内容。我们利用2016年至2019年的全国再入院数据库进行了一项回顾性队列研究，对AHA进行了描述性分析，包括对30天再入院率的估计和再入院时的初步诊断。入院的1450例AHA患者中，803例（55.4%）为男性，入院时中位年龄为73岁。约21%有潜在的实体恶性肿瘤，3.9%有血液恶性肿瘤，13.5%有自身免疫性疾病。急性心肌梗死发生率为9.5%，弥散性血管内凝血发生率为1.2%，颅内出血发生率为1.5%，缺血性卒中发生率为2.5%，静脉血栓栓塞发生率为4.4%。入院患者出血发生率为30.2%，其中27%需要输血。住院时间中位数为7天，指数入院期间有101例死亡，住院死亡率为7%。老年组的死亡率为8%，高于65岁组的3% （P = .03）。30天再入院率为27%，再入院期间死亡率为10.8%。感染后出血是再入院的最常见原因。AHA患者出血和血栓并发症发生率高，出血和感染是30天再入院率升高的主要原因，表明显著的治疗相关毒性和疾病复发。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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Blood Vessels, Thrombosis & Hemostasis

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