具有单克隆免疫球蛋白沉积的扩散性淋巴结膜炎的IgM变体:一个病例系列
Lihong Bu1, Anthony M Valeri2, Samar M Said3
1Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota.
概括
具有单克隆性免疫球蛋白沉积的增殖性淋巴结膜炎 - - IgM 变体 (PGNMID-IgM) 主要影响患有关键单克隆性淋巴结膜病变 (MGRS) 的老年男性. 的存活率受到保护,但患者的存活率是有利的,移植复发率很高.
科学领域:
- 腎臟病學 (nephrology) 是一種醫學.
- 免疫病理学 免疫病理学
- 血液学 血液学 血液学
背景情况:
- 具有单克隆性免疫球蛋白沉积 (PGNMID) 的增殖性血球炎通常涉及IgG沉积.
- 该IgM变体 (PGNMID-IgM) 很少见,主要来自病例报告或与B细胞疾病相关的数据有限.
研究的目的:
- 描述PGNMID-IgM的临床病理学特征.
- 评估PGNMID-IgM.患者的结局和生存期.
主要方法:
- 从病理档案中确定了23名PGNMID-IgM患者的回顾性病例系列.
- 定义的PGNMID-IgM由质核突炎与质颗粒单型IgM沉积物,不包括其他特定条件.
- 分析了临床表现,实验室发现,脏活检结果,治疗和患者/脏存活率.
主要成果:
- 该队列包括老年男性 (平均年龄72岁),他们呈现蛋白尿,血液和功能下降.
- 所有病例都有性意义的单克隆性甘马病变 (MGRS);与SPEP/SIF相比,MALDI-TOF改善了性IgM的检测.
- 脏活检显示了膜增殖性球腺炎与非组织的单型IgM和C3沉积;脏存活时间为44个月,患者存活时间为158个月,移植复发率高.
结论:
- PGNMID-IgM主要影响患有MGRS的老年男性,并呈现出特有的病理.
- 循环病性IgM的诊断产量通过MALDI-TOF质谱学得到了增强.
- 的存活率受到保护,移植后复发是常见的,尽管患者的整体存活率是有利的.
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