Spinal Stenosis: An Emerging Complication of Ageing in People With Haemophilia

IF 3 2区 医学 Q2 HEMATOLOGY
Haemophilia Pub Date : 2025-07-29 DOI:10.1111/hae.70089
Claire Kelly, Mark McGowan, Niamh Larkin, Jake M. Mc Donnell, Anne-Marije Hilshof, Mary Byrne, Catherine Bergin, Aine O'Gara, Kevin Ryan, Mairead O'Donovan, Niamh O'Connell, Keith Synnott, Joseph S. Butler, Stacey Darwish, Brian O'Mahony, Megan Kennedy, Peter L. Turecek, James S. O'Donnell, John Gormley, Michelle Lavin
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Abstract

Introduction

Advances in haemophilia care have brought the challenges of ageing for people with haemophilia (PWH) to the forefront. Age-related spinal degeneration may result in spinal stenosis; however, the rates in PWH are unknown. We sought to systematically review Irish PWH to address this gap in the current literature.

Methods

Clinical and radiological notes of all patients ≥40 years old (yo) registered with severe or moderate haemophilia A or B were reviewed, recording Haemophilia Joint Health Scores (HJHS), radiological imaging and orthopaedic/pain interventions.

Results

Of 100 males included with moderate or severe haemophilia, 13% had radiologically confirmed symptomatic spinal stenosis (reported rates 4% in the general population aged >60 yo). Persons with stenosis were older (median age 69yo vs. 55 yo, p = 0.004) with similar rates observed between those with moderate and severe haemophilia (4/35, 11.4% vs. 9/65, 13.8%). HJHS did not differ between those with and without stenosis (median 30 vs. 35, p = 0.6). On regression analysis, only age >60 yo was associated with an increased likelihood of spinal stenosis; severity of haemophilia (moderate vs. severe) was not significantly associated.

Conclusions

These data identify symptomatic spinal stenosis as a novel complication of ageing for PWH. Spinal stenosis rates were higher than expected for age in comparison to reported rates in the general population. Current joint assessments fail to capture spinal pathology, highlighting limitations of HJHS in older PWH. Increased awareness amongst PWH and health care providers of spinal stenosis is directly required; however, optimal management strategies for PWH with established stenosis are yet to be defined.

Abstract Image

椎管狭窄:血友病患者衰老的新并发症。
导言:血友病护理的进步使血友病患者(PWH)老龄化的挑战成为人们关注的焦点。年龄相关性脊柱退变可导致椎管狭窄;然而,PWH的发病率尚不清楚。我们试图系统地回顾爱尔兰PWH,以解决当前文献中的这一差距。方法:回顾所有年龄≥40岁的重度或中度血友病A或B患者的临床和放射学记录,记录血友病关节健康评分(HJHS)、放射影像学和矫形/疼痛干预。结果:在100名患有中度或重度血友病的男性中,13%的人有影像学证实的症状性椎管狭窄(在60岁至60岁的普通人群中报道的比例为4%)。狭窄患者年龄较大(中位年龄69岁vs. 55岁,p = 0.004),中度和重度血友病患者的发生率相似(4/35,11.4% vs. 9/65, 13.8%)。有和没有狭窄的患者HJHS无差异(中位数30 vs 35, p = 0.6)。在回归分析中,只有年龄在50 - 60岁之间与椎管狭窄的可能性增加有关;血友病的严重程度(中度与重度)无显著相关性。结论:这些数据确定症状性椎管狭窄是PWH衰老的新并发症。与一般人群中报道的椎管狭窄率相比,年龄的椎管狭窄率高于预期。目前的联合评估未能捕获脊柱病理,突出了HJHS在老年PWH中的局限性。直接需要提高PWH和保健提供者对椎管狭窄的认识;然而,对于已确定狭窄的PWH的最佳管理策略尚未确定。
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
Haemophilia
Haemophilia 医学-血液学
CiteScore
6.50
自引率
28.20%
发文量
226
审稿时长
3-6 weeks
期刊介绍: Haemophilia is an international journal dedicated to the exchange of information regarding the comprehensive care of haemophilia. The Journal contains review articles, original scientific papers and case reports related to haemophilia care, with frequent supplements. Subjects covered include: clotting factor deficiencies, both inherited and acquired: haemophilia A, B, von Willebrand''s disease, deficiencies of factor V, VII, X and XI replacement therapy for clotting factor deficiencies component therapy in the developing world transfusion transmitted disease haemophilia care and paediatrics, orthopaedics, gynaecology and obstetrics nursing laboratory diagnosis carrier detection psycho-social concerns economic issues audit inherited platelet disorders.
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