Regular assessment of serum vascular endothelial growth factor levels to monitor POEMS syndrome,Neurological Sciences

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Regular assessment of serum vascular endothelial growth factor levels to monitor POEMS syndrome
Neurological Sciences ( IF 2.7 ) Pub Date : 2023-09-13 , DOI: 10.1007/s10072-023-07064-5
Francesco Gentile _{1,

2} , Fabrizia Terenghi ₁ , Pietro Emiliano Doneddu _{1,

3} , Alberto De Lorenzo _{1,

2} , Claudia Giannotta ₁ , Andrea Giordano _{1,

2} , Rita Mazza ₄ , Andrea Nozza ₄ , Eduardo Nobile-Orazio _{1,

5}

Affiliation

Background

To investigate the utility of regular serum VEGF (sVEGF) levels assessment in the monitoring of POEMS syndrome.

Methods

We retrospectively reviewed data of 30 patients with POEMS syndrome whose sVEGF was tested regularly every 6 months. sVEGF levels after treatment were measured and correlated with disability (Overall Neuropathy Limitations Scale, ONLS), clinical impairment (measured with the modified Clinical Response Evaluation Scale, mCRES), and relapse-free survival. The ability of sVEGF to predict disease flares during remission and refractory disease was also analysed.

Results

Patients with normalised serum VEGF levels (< 1000 pg/ml) at 6 months showed prolonged relapse-free survival (at 3-year 94% for complete VEGF response, 57% partial, 0% none, p < 0.001) and greater later clinical improvement (median ΔmCRES complete VEGF response -5 vs partial -4, p = 0.019, and vs no VEGF response -2, p = 0.006). After remission, the sensitivity of 6-month sVEGF monitoring in predicting clinical relapse was 58% with a specificity of 100%. In patients refractory to treatment, the sensitivity in predicting further clinical worsening was 15%. In addition, in 25% of the patients in remission and 16% of those refractory to therapy, sVEGF levels only increased at the time of relapse.

Conclusions

Regular sVEGF assessment is a valid biomarker in the prediction of disease reactivation in POEMS syndrome and was particularly useful during the phase of remission.

中文翻译：

定期评估血清血管内皮生长因子水平以监测 POEMS 综合征

背景

探讨定期血清 VEGF (sVEGF) 水平评估在监测 POEMS 综合征中的实用性。

方法

我们回顾性分析了 30 名 POEMS 综合征患者的数据，每 6 个月定期检测一次 sVEGF。治疗后测量 sVEGF 水平，并将其与残疾（总体神经病变限制量表，ONLS）、临床损伤（使用改良的临床反应评估量表，mCRES 测量）和无复发生存率相关联。还分析了 sVEGF 预测缓解期和难治性疾病期间疾病发作的能力。

结果

6 个月时血清 VEGF 水平正常化 (< 1000 pg/ml) 的患者表现出较长的无复发生存期（3 年时 VEGF 完全缓解率为 94%，部分缓解率为 57%，无缓解率为 0%， p < 0.001），并且后期临床表现更高改善（中位 ΔmCRES VEGF 完全反应 -5 与部分 -4， p = 0.019，和无 VEGF 反应 -2， p = 0.006）。缓解后，6个月的sVEGF监测预测临床复发的敏感性为58%，特异性为100%。对于难治性治疗的患者，预测临床进一步恶化的敏感性为 15%。此外，在 25% 的缓解期患者和 16% 的治疗难治性患者中，sVEGF 水平仅在复发时升高。