游离轻链和重/轻链在监测 POEMS 患者中的应用。

Free light chains and heavy/light chains in monitoring POEMS patients.

出版信息

Clin Chem Lab Med. 2016 Jun 1;54(6):1065-71. doi: 10.1515/cclm-2015-0910.

DOI:10.1515/cclm-2015-0910

Abstract

BACKGROUND

POEMS syndrome is defined by Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy and Skin changes. The vascular endothelial growth factor (VEGF) appears to play a key role in the pathogenesis of the syndrome, and its concentrations are deemed to correlate to disease activity. The aim of the present study was to verify whether other biochemical markers including serum free light chains (FLC) and heavy/light chains (HLC) would be of value in monitoring POEMS patients.

METHODS

Fifty-three serum samples were collected from seven POEMS patients at diagnosis and during a follow-up period (range 14-56 months). VEGF was measured using an ELISA method, while FLC and HLC concentrations were measured using Binding Site reagents on a BNII (Siemens) nephelometer.

RESULTS

At diagnosis all patients presented high VEGF concentrations, while the κ/λFLC ratio (FLCr) was within the reference range. Four patients had abnormal HLC, HLCκ/HLCλ (HLCr) and FLC values. The relationship between the trend of VEGF and both HLC and FLC during the follow-up was analysed by means of Cohen's κ coefficient. VEGF and HLC values displayed a significant κ-Cohen (0.537, p=0.002) in all chemotherapy-responder patients while in non-responders it did not. Conversely, in both responders and non-responders, VEGF and FLC values did not attain a significance on κ-Cohen analysis. In three out of four responders HLCr values increased, thus reflecting an improved clinical condition.

CONCLUSIONS

The findings made in the present study indicate that HLC, either as intact immunoglobulin or as HLCr, may provide useful information, particularly in identifying responders and confirm that the role of FLC is unreliable in monitoring patients with POEMS syndrome.

摘要

背景

POEMS 综合征的定义为多发性神经病、器官肿大、内分泌病、单克隆丙种球蛋白病和皮肤改变。血管内皮生长因子（VEGF）似乎在该综合征的发病机制中发挥关键作用，其浓度被认为与疾病活动相关。本研究的目的是验证其他生化标志物，包括血清游离轻链（FLC）和重链/轻链（HLC），在监测 POEMS 患者方面是否具有价值。

方法

从 7 名 POEMS 患者的 53 份血清样本中收集了诊断时和随访期间（范围 14-56 个月）的样本。使用 ELISA 法测量 VEGF，使用 Binding Site 试剂在 BNII（西门子）散射光度计上测量 FLC 和 HLC 浓度。

结果

所有患者在诊断时均表现出高 VEGF 浓度，而 κ/λFLC 比值（FLCr）在参考范围内。4 名患者的 HLC、HLCκ/HLCλ（HLCr）和 FLC 值异常。通过 Cohen's κ 系数分析了随访期间 VEGF 与 HLC 和 FLC 趋势之间的关系。在所有化疗反应者中，VEGF 和 HLC 值之间存在显著的 κ-Cohen（0.537，p=0.002），而在非反应者中则没有。相反，在反应者和非反应者中，VEGF 和 FLC 值在 κ-Cohen 分析中均未达到显著水平。在 4 名反应者中有 3 名 HLCr 值升高，反映出临床状况改善。