一个沃尔登斯特罗姆巨球蛋白血症病例呈现为恶化的神经病变
Meher Binte Ali1, Zunera Huda2, Marcus Edward Hendricks3
1Internal Medicine, University of Maryland Medical Center, Baltimore, USA.
Cureus
|October 3, 2025
概括
沃尔登斯特罗姆巨球蛋白血症 (WM) 可以表现为神经病变,模仿其他疾病. 早期诊断和治疗对于管理这种罕见的B细胞淋巴瘤和预防神经损伤至关重要.
科学领域:
- 血液学 血液学 血液学
- 神经学 神经学
- 在瘤学瘤学.
背景情况:
- 沃尔登斯特罗姆巨型球蛋白血症 (WM) 是一种罕见的淋巴细胞性淋巴瘤.
- 它涉及单克隆IgM分泌B细胞的增殖,经常呈现出贫血,脏/肝脏扩大和淋巴结.
研究的目的:
- 描述WM呈现为渐进性神经病变的病例.
- 突出诊断挑战和考虑WM在非典型神经病变病例的重要性.
主要方法:
- 病例报告详细介绍了一名最初因退行性脊髓疾病接受治疗的患者.
- 诊断工作包括血清蛋白电泳 (SPEP),外围神经活检和骨髓活检.
- 治疗涉及bendamustine-rituximab,其次是扎努布鲁丁尼布.
主要成果:
- 这位患者出现了最初误诊的神经病变.
- 诊断证实了WM与MYD88 L265P突变,单克隆M尖峰和内性IgM沉积.
- 用本达穆斯丁-利图西马布治疗导致部分稳定,但神经病变复发,需要扎努布鲁丁尼布.
结论:
- 与WM相关的偏蛋白质性神经病变可能是诊断上复杂的.
- 在非典型神经病变的差异诊断中考虑WM对于及时干预至关重要.
- 及时诊断和治疗可以帮助缓解WM患者不可逆转的神经损伤.
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