β-地中海贫血症骨髓脂肪含量和成分：一项使用 H-MRS 的研究。

Marrow Fat Content and Composition in β-Thalassemia: A Study using H-MRS.

机构信息

Department of Biomedical Imaging, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia.

Haematology Unit, Department of Medicine, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia.

出版信息

J Magn Reson Imaging. 2021 Jan;53(1):190-198. doi: 10.1002/jmri.27294. Epub 2020 Aug 15.

DOI:10.1002/jmri.27294

PMID:33237616

Abstract

BACKGROUND

β-thalassemia is a genetic disease that causes abnormal production of red blood cells (ineffective erythropoiesis, IE). IE is a condition known to change bone marrow composition.

PURPOSE

To evaluate the effect of IE on the marrow fat content and fat unsaturation levels in the proximal femur using H-MRS.

STUDY TYPE

Prospective.

SUBJECTS

Twenty-three subjects were included in this study, seven control and 16 β-thalassemia subjects.

FIELD STRENGTH/SEQUENCE: 3.0T; stimulated echo acquisition Mode (STEAM); magnetic resonance spectroscopy (MRS) sequence.

ASSESSMENT

Multiecho MRS scans were performed in four regions of the proximal left femur of each subject, that is, diaphysis, femoral neck, femoral head, and greater trochanter. The examined regions were grouped into red (diaphysis and femoral neck) and yellow marrow regions (femoral head and greater trochanter).

STATISTICAL TESTS

The Jonckheere-Terpstra test was used to evaluate the impact of increasing disease severity on bone marrow fat fraction (BMFF), marrow conversion index, and fat unsaturation index (UI). Pairwise comparison analysis was performed when a significant trend (P < 0.05) was found. K-means clustering analysis was used to examine the clusters observed when BMFF in the red and yellow regions were studied (diaphysis against greater trochanter).

RESULTS

BMFF showed a significant decreasing trend with increasing disease severity in both red (T = 109.00, z = -4.414, P < 0.05) and yellow marrow regions (T = 108.00, z = -4.438, P < 0.05). The opposite trend was observed in UI in both bone marrow regions (red marrow: T = 180.5, z = 3.515, P < 0.05; yellow marrow: T = 155.0, z = 2.282, P = 0.05). Three distinct forms of marrow adipogenesis were found when plotting BMFF diaphysis against BMFF greater trochanter: 1) normal (centroid: 80.4%, 66.6%), 2) partial disruption (centroid: 51.1%, 16.6%), and 3) total disruption (centroid: 2.6%, 1.6%).

DATA CONCLUSION

β-thalassemia is associated with decreased marrow fat, and increased marrow fat unsaturation level.

LEVEL OF EVIDENCE

2 TECHNICAL EFFICACY STAGE: 3.

摘要

背景

β-地中海贫血是一种导致红细胞生成异常（无效性红细胞生成，IE）的遗传性疾病。IE 是一种已知会改变骨髓成分的病症。

目的

使用 H-MRS 评估 IE 对近端股骨骨髓脂肪含量和脂肪不饱和水平的影响。

研究类型

前瞻性。

受试者

本研究共纳入 23 名受试者，其中 7 名为对照组，16 名为β-地中海贫血组。

磁场强度/序列：3.0T；激发回波采集模式（STEAM）；磁共振波谱（MRS）序列。

评估

对每位受试者的左侧股骨近端四个区域（骨干、股骨颈、股骨头和大转子）进行多回波 MRS 扫描。检查区域分为红骨髓区（骨干和股骨颈）和黄骨髓区（股骨头和大转子）。

统计学检验

使用 Jonckheere-Terpstra 检验评估疾病严重程度增加对骨髓脂肪分数（BMFF）、骨髓转化率指数和脂肪不饱和指数（UI）的影响。当发现显著趋势（P<0.05）时，进行两两比较分析。当研究红骨髓和黄骨髓区域的 BMFF 时，使用 K-均值聚类分析来检查观察到的聚类（骨干与大转子）。

结果

在红骨髓（T=109.00，z=-4.414，P<0.05）和黄骨髓（T=108.00，z=-4.438，P<0.05）区域，BMFF 随着疾病严重程度的增加呈显著下降趋势。在两种骨髓区域（红骨髓：T=180.5，z=3.515，P<0.05；黄骨髓：T=155.0，z=2.282，P=0.05），UI 呈相反的趋势。当绘制骨干 BMFF 与大转子 BMFF 时，发现三种不同形式的骨髓脂肪生成：1）正常（质心：80.4%，66.6%），2）部分破坏（质心：51.1%，16.6%）和 3）完全破坏（质心：2.6%，1.6%）。