病例报告:POEMS综合征是多个孤独血细胞瘤的次要症状,并由高甘油三血症复杂化
Wenchao Chen1,2, Fang Xue1, Na Wang3
1Department of Neurology, The Second Hospital of Shanxi Medical University, Taiyuan, China.
Frontiers in medicine
|January 21, 2026
概括
作为一种罕见疾病,POEMS综合征可以在没有可检测的M蛋白的情况下进行诊断. 这一案例凸显了在患有多系统性问题和高甘油三血症的患者中考虑POEMS综合征的重要性,即使没有M蛋白.
科学领域:
- 内分泌学 在内分泌学.
- 神经学 神经学
- 血液学 血液学 血液学
背景情况:
- POEMS综合征 (多神经病,有机巨变,内分泌病,M蛋白,皮肤变化) 是一种罕见的多系统性疾病.
- 诊断通常依赖于检测单克隆蛋白 (M-蛋白),导致M-蛋白阴性病例的潜在误诊.
研究的目的:
- 要呈现一种在没有可检测的M蛋白的情况下诊断的POEMS综合征病例.
- 强调在非典型的呈现中考虑POEMS综合征的重要性.
主要方法:
- 对患有渐进性神经病变,皮肤变化,,甲状腺功能低下和高甘油三血症的患者进行诊断评估.
- 利用血清和骨髓研究,成像和单一血细胞瘤的病理评估.
主要成果:
- 通过检测兰巴光链受限单独血细胞瘤,尽管阴性血清/骨髓M蛋白,但确立了POEMS综合征的诊断.
- 患者在基于博雷佐米布的化疗后显著改善.
结论:
- 在多系统参与和严重的高甘油三血症的情况下,即使没有M蛋白,也应该怀疑POEMS综合征.
- 早期识别非典型的POEMS综合征表现对于及时治疗和改善结果至关重要.
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