带有纤维状沉积物的淋巴细胞病变
Pavlina Dzekova-Vidimliski1,2, Vlatko Karanfilovski1,2, Igor G Nikolov1,2
11University Hospital of Nephrology, Skopje, RN Macedonia.
概括
纤维状和免疫性质结晶病是一种罕见的脏疾病,其特点是微纤维细胞沉积. 诊断依赖于电子显微镜,通过微纤维素的大小和排列来区分它们,目前没有治疗指南.
科学领域:
- 腎臟病學 (nephrology) 是一種醫學專業.
- 病理学 病理学 病理学
- 电子显微镜电子显微镜
背景情况:
- 凝结体病包括细胞外纤维的沉积,根据刚果红色染色分类为粉样化或非粉样化类型.
- 非粉样菌性血球蛋白病变进一步划分为免疫球蛋白衍生的和非免疫球蛋白衍生的类别.
- 纤维状血小球病 (FGn) 和免疫状血小球病 (ITG) 是一种罕见的免疫球蛋白衍生的疾病.
研究的目的:
- 根据超结构形态学来区分纤维状质细胞病变 (FGn) 和免疫性质细胞病变 (ITG).
- 突出这些罕见的质细胞病变的临床表现和诊断挑战.
主要方法:
- 刚果红色染色以区分粉样性粉症和非粉样性粉样性粉病.
- 电子显微镜分析FGn和ITG中的微纤维特征 (大小,排列,光线存在).
- 对患者表现和疾病进展的临床数据审查.
主要成果:
- FGn微纤维是随机排列的,直径小于30nm.
- ITG微纤维的长度大于30nm,具有可见的光线 (微管),并排列成平行束.
- 患有性范围蛋白尿症,血尿症,高血压和进展性慢性病的患者.
结论:
- 电子显微镜对于区分FGn和ITG,基于不同的微纤维超结构,至关重要.
- 纤维状-免疫性状球膜病变表现为严重的功能障碍,缺乏确定的治疗指南.
- 如果与血液学疾病相关联,免疫性球症可能需要克隆导向治疗.
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