威普尔病最初呈现为类风湿关节炎患者的脑关节上淋巴瘤病:一个诊断挑战
Sara E Amin1, Swati Gite1, Audrey Wanger1
1Department of Pathology and Laboratory Medicine, The University of Texas Health Science Center at Houston, Houston, TX, USA.
International journal of surgical pathology
|May 21, 2025
概括
惠普尔病是一种罕见的Tropheryma whipplei感染,具有非特异性症状的诊断挑战. 通过分子检测和PAS染色的早期诊断对于有效治疗和预防严重后果至关重要.
科学领域:
- 传染性疾病 传染性疾病
- 病理学 病理学 病理学
- 类风湿病学 类风湿病学
背景情况:
- 惠普尔病是一种罕见的,慢性,系统性感染,由Tropheryma whipplei引起.
- 它经常表现为非特异性症状,模仿其他疾病,并造成诊断困难.
- 免疫受损的个体可能会表现出不典型的表现,包括淋巴腺病和白细胞病.
研究的目的:
- 为了突出怀普尔病在免疫受损患者的诊断挑战.
- 强调在患有无法解释的淋巴腺病和全身症状的患者中考虑惠普尔病的重要性.
- 为了强调特定的诊断方法在确认Tropheryma whipplei感染中的作用.
主要方法:
- 一个61岁的男性患有类风湿性关节炎和CIDP的病例介绍,呈现出白细胞体和淋巴腺病变.
- 诊断工作包括PET扫描,淋巴结活检与组织病理学,免疫组织化学,特殊染料 (Grocott-Gömöri, PAS),电子显微镜和PCR.
- 考虑的差异性诊断包括淋巴增殖性疾病,沙尔科毒症和其他感染.
主要成果:
- 活检揭示了泡状的囊细胞透物与PAS阳性,抗扩散酶的包容物.
- 免疫组织化学检测结果对常见的瘤标志物 (S100,CD1a,BRAF V600E) 是负的.
- 电子显微镜和PCR证实了Tropheryma whipplei的存在,确定了诊断.
结论:
- 惠普尔病可以在免疫功能低下的患者中呈现异常,模仿淋巴瘤或肉类瘤.
- 定期酸-Schiff (PAS) 染色和Tropheryma whipplei的分子检测对于诊断至关重要.
- 迅速诊断和治疗惠普尔病至关重要,因为它是一种可治愈的,但如果不治疗,可能致命的疾病.
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