血友病严重程度对骨密度及骨折风险的影响

IF 3.4 3区医学 Q2 HEMATOLOGY

Research and Practice in Thrombosis and Haemostasis Pub Date : 2024-11-01 DOI:10.1016/j.rpth.2024.102624

Pia Ransmann , Jamil Hmida , Marius Brühl , Frank Alexander Schildberg , Georg Goldmann , Johannes Oldenburg , Max Jaenisch , Fabian Tomschi , Thomas Hilberg , Andreas Christian Strauss

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引用次数: 0

摘要

背景：有证据表明血友病患者经常受到低骨密度（BMD）的影响。评估血友病严重程度与骨质疏松症发生之间关系的数据缺乏。目的：本前瞻性队列研究旨在评估血友病严重程度对骨密度的影响，并调查骨小梁评分（TBS）和骨折风险（FRAX）。方法采用双x线吸收仪对255例血友病患者的骨密度、TBS和FRAX进行了前瞻性队列研究。采用国际临床密度测定学会指南进行分类：骨质疏松症（T-score < - 2.5）、骨质减少症（T-score < - 1.0）、正常（T-score > - 1.0）。年龄小于50岁且z评分为<；−2.0的患者被认为低于年龄的预期范围。结果255例血友病患者（轻度52例，中度53例，重度150例），年龄43±15岁（平均±SD），骨密度降低63.1%。甚至11.9%的50岁血友病患者被归类为低于预期年龄范围。颈部骨密度随严重程度呈线性下降(轻度：0.907±0.229，中度：0.867±0.131，重度：0.799±0.143；P = 0.01)。n = 178（81.3%）例TBS归为“正常”，平均值为1.403±0.136，严重程度之间无差异（P = 0.54）。FRAX为4.4%±3.0%。TBS调整后为2.8%±3.7%。结论血友病患者骨密度降低的比例为63.1%，且与血友病严重程度有关。然而，大体正常的TBS意味着骨骼的微结构似乎没有受到影响。建议将骨质疏松筛查，包括TBS分析，纳入血友病患者的综合诊断工作中，特别是随着年龄的增长。

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The influence of severity of hemophilia on bone mineral density and fracture risk

Background

Evidence states that persons with hemophilia are frequently affected by low bone mineral density (BMD). Data assessing the relationship between severity of hemophilia and occurrence of osteoporosis are lacking.

Objectives

This prospective cohort study aimed to assess the impact of hemophilia severity on BMD and to investigate trabecular bone score (TBS) and fracture risk (FRAX).

Methods

This prospective cohort study evaluated the BMD, TBS, and FRAX in 255 persons with hemophilia using dual x-ray absorptiometry. The International Society for Clinical Densitometry guidelines were used for classification: osteoporosis (T-score <−2.5), osteopenia (T-score <−1.0), normal (T-score >−1.0). Patients younger than 50 years of age with a Z-score of <−2.0 were considered below the expected range for age.

Results

Of 255 persons with hemophilia (mild: n = 52, moderate: n = 53, severe: n = 150) aged 43 ± 15 years (mean ± SD), 63.1% showed reduced BMD. Even 11.9% of persons with hemophilia aged <50 years were classified as below the expected range for age. Neck BMD decreased linearly with severity (mild: 0.907 ± 0.229, moderate: 0.867 ± 0.131, severe: 0.799 ± 0.143; P = .01). TBS was classified as “normal” in n = 178 (81.3%) with a mean value of 1.403 ± 0.136, and there were no differences between severity levels (P = .54). The FRAX was 4.4% ± 3.0%. After adjustment of TBS, it was 2.8% ± 3.7%.

Conclusion

The present study shows that BMD is decreased in 63.1% of persons with hemophilia also depending on the severity of hemophilia. However, the largely normal TBS implies that the microarchitecture of the bone does not seem to be affected. It is recommended to include osteoporosis screening, including TBS analysis, in the comprehensive diagnostic work-up of persons with hemophilia, especially as they age.

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