血清游离轻链检测在AL淀粉样变性患者中的诊断及预后价值
Diagnostic and prognostic utility of the serum free light chain assay in patients with AL amyloidosis.
作者信息
Morris K L, Tate J R, Gill D, Kennedy G, Wellwood J, Marlton P, Bird R, Mills A K, Mollee P
机构信息
Department of Clinical and Laboratory Haematology, Queensland Health Pathology Service, Princess Alexandra Hospital, Woolloongabba, Queensland, Australia.
出版信息
Intern Med J. 2007 Jul;37(7):456-63. doi: 10.1111/j.1445-5994.2007.01368.x.
BACKGROUND
Organ dysfunction in AL amyloidosis is related to the production and deposition of amyloidogenic monoclonal light chains. These pathological light chains can now be quantified using the recently developed serum free light chain assay.
METHODS
We retrospectively reviewed 31 patients with AL amyloidosis to determine the frequency of abnormal free light chain assay results at diagnosis and whether changes in the serum free light chain assay predict outcome after therapy.
RESULTS
An abnormal free light chain assay was found in 30 of 31 patients (97%) at the time of diagnosis. In the subset of our patients who received treatment for AL amyloidosis, a >50% reduction of the pathological free light chain following treatment was shown to predict improved overall survival. In our series of analyses, achievement of greater magnitudes of reduction of the free light chain result did not appear to provide additional prognostic information, nor did the baseline free light chain result predict outcome.
CONCLUSION
Our findings support the use of the free light chain assay in the diagnostic work-up of patients with suspected AL amyloidosis, and also as a sensitive biomarker of response to therapy.
背景
AL淀粉样变性中的器官功能障碍与淀粉样变性单克隆轻链的产生和沉积有关。现在可以使用最近开发的血清游离轻链检测法对这些病理性轻链进行定量。
方法
我们回顾性分析了31例AL淀粉样变性患者，以确定诊断时游离轻链检测结果异常的频率，以及血清游离轻链检测结果的变化是否能预测治疗后的预后。
结果
31例患者中有30例（97%）在诊断时游离轻链检测结果异常。在我们接受AL淀粉样变性治疗的患者亚组中，治疗后病理性游离轻链降低>50%可预测总体生存率提高。在我们的一系列分析中，游离轻链结果降低幅度更大似乎并不能提供额外的预后信息，基线游离轻链结果也不能预测预后。
结论
我们的研究结果支持在疑似AL淀粉样变性患者的诊断检查中使用游离轻链检测法，也支持将其作为治疗反应的敏感生物标志物。
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