Retroperitoneal and lower extremities muscle bleeding in acquired haemophilia A (AHA): Risk factors and implications in disability and survival

IF 2.3 3区医学 Q2 HEMATOLOGY

European Journal of Haematology Pub Date : 2024-07-31 DOI:10.1111/ejh.14279

José Pardos-Gea, Olga Benítez

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引用次数: 0

Abstract

Aim

To assess risk factors of retroperitoneal and lower extremity musculoskeletal bleed in acquired haemophilia (AHA) and perform an objective assessment of disability and influence on survival.

Methods

We included 49 patients with AHA from November 2017 to May 2023. The occurrence of any retroperitoneal or/and lower extremities bleeding manifestation was investigated. On clinical follow-up, we search for compressive femoral neuropathy and quadriceps amyotrophy. The lower extremity functional scale (LEFS) was carried out one year after the last bleeding event in all AHA patients.

Results

A 61.2% of patients in our AHA cohort presented with any retroperitoneal and/or lower extremities musculoskeletal manifestation. Those patients had higher percentage of major bleeding EACH2/ISTH criteria (90% vs. 57%, p = .01), needs of blood transfusions (86% vs. 57% of patients, p = .03), and haemostatic by-pass products (90% vs. 63%, p = .02). Hypertension (HR 2.6, 95% CI 1.1–5.9, p = .02), presence of autoimmune disease (HR 13, 95% CI 1.7–99, p = .01), and inhibitor level > 20 BU (HR 2.6 95% CI 1.0–6.8, p = .04) significantly predicted retroperitoneal/lower extremities clinical manifestations. Most frequent sequelae were quad atrophy (30.6%) and femoral nerve palsy (20.4%). Quad atrophy and LEFS scores under 50 were associated with increased mortality (HR 3, 95% CI 1.1–8.6 and HR 12, 95% CI 3.3–45, respectively).

Conclusion

AHA with retroperitoneal/lower extremities bleeding involvement is of greater severity and shows high disability and worst survival outcomes. Quadriceps atrophy and LEFS scale scoring under 50 predicted mortality in our AHA patients.

查看原文本刊更多论文

获得性血友病 A（AHA）腹膜后和下肢肌肉出血：风险因素及其对残疾和存活的影响。

目的：评估获得性血友病（AHA）腹膜后和下肢肌肉骨骼出血的风险因素，并对残疾情况和对生存的影响进行客观评估：我们纳入了2017年11月至2023年5月的49名AHA患者。调查了任何腹膜后或/和下肢出血表现的发生情况。在临床随访中，我们寻找压迫性股神经病变和股四头肌肌萎缩。在最后一次出血事件发生一年后，我们对所有AHA患者进行了下肢功能量表（LEFS）测试：结果：在我们的 AHA 队列中，61.2% 的患者有腹膜后和/或下肢肌肉骨骼表现。这些患者符合 EACH2/ISTH 大出血标准（90% 对 57%，P = 0.01）、需要输血（86% 对 57%，P = 0.03）和使用止血产品（90% 对 63%，P = 0.02）的比例较高。高血压（HR 2.6，95% CI 1.1-5.9，p = .02）、自身免疫性疾病（HR 13，95% CI 1.7-99，p = .01）和抑制剂水平大于 20 BU（HR 2.6，95% CI 1.0-6.8，p = .04）可显著预测腹膜后/下肢临床表现。最常见的后遗症是四肢萎缩（30.6%）和股神经麻痹（20.4%）。四肢萎缩和LEFS评分低于50与死亡率增加有关（分别为HR 3，95% CI 1.1-8.6和HR 12，95% CI 3.3-45）：结论：腹膜后/下肢出血受累的 AHA 患者病情更为严重，致残率高，存活率最差。股四头肌萎缩和LEFS量表评分低于50分预示着AHA患者的死亡率。

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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来源期刊

European Journal of Haematology 医学-血液学

CiteScore

5.50

自引率

0.00%

发文量

168

审稿时长

4-8 weeks

期刊介绍： European Journal of Haematology is an international journal for communication of basic and clinical research in haematology. The journal welcomes manuscripts on molecular, cellular and clinical research on diseases of the blood, vascular and lymphatic tissue, and on basic molecular and cellular research related to normal development and function of the blood, vascular and lymphatic tissue. The journal also welcomes reviews on clinical haematology and basic research, case reports, and clinical pictures.