Prognostic value of serum heavy/light chain ratios in patients with POEMS syndrome

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• 作者：Chen Wang ; Wei Su ; Qian-Qian Cai ; Hao Cai ; Wei Ji ; Qian Di ; Ming-Hui Duan ; Xin-Xin Cao ; Dao-Bin Zhou and Jian Li
• 刊名：European Journal of Haematology
• 出版年：2016
• 出版时间：July 2016
• 年：2016
• 卷：97
• 期：1
• 页码：48-54
• 全文大小：319K
• ISSN：1600-0609

文摘

POEMS syndrome is a rare plasma cell dyscrasia. Serum concentrations of the monoclonal protein in this disorder are typically low, and inapplicable to monitor disease activity in most cases, resulting in limited practical and prognostic values. Novel immunoassays measuring isotype-specific heavy/light chain (HLC) pairs showed its utility in disease monitoring and outcome prediction in several plasma cell dyscrasias. We report results of HLC measurements in 90 patients with POEMS syndrome. Sixty-six patients (73%; 95% confidence interval, 63–82%) had an abnormal HLC ratio at baseline. It could stratify the risk of disease relapse and was strongly associated with worse progression-free survival in a multivariate analysis (P = 0.021; hazard ratio [HR] 6.89, 95% CI 1.34–35.43). After therapy, HLC ratios improved, with 43 patients (48%) remaining abnormal. The post-therapeutic HLC ratio, if abnormal, also remained as an independent prognostic factor associated with worse progression-free survival (P = 0.019; HR 4.30, 95% CI 1.27–14.56). These results suggest the prognostic utility of HLC ratios in clinical management of POEMS patients.