ISSN 1006-298X      CN 32-1425/R

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肾脏病与透析肾移植杂志 ›› 2024, Vol. 33 ›› Issue (1): 87-91.DOI: 10.3969/j.issn.1006-298X.2024.01.017

• 肾活检 • 上一篇    下一篇

伴多核巨细胞浸润的轻链型肾淀粉样变性

  

  • 出版日期:2024-02-28 发布日期:2024-02-27

Renal light chain amyloidosis with multinucleated giant cells

  • Online:2024-02-28 Published:2024-02-27

摘要: 64岁男性患者,临床表现为肾病综合征,无高血压及肾功能损害,血免疫固定电泳检查示λ型IgA单克隆免疫球蛋白条带,血游离轻链检查示κ/λ比值下降;骨髓流式细胞学见4.52%单克隆增生的浆细胞,外院肾活检诊断轻链型肾淀粉样变性。患者化疗后临床达非常好的部分缓解,经重复肾活检后诊断为罕见的伴多核巨细胞浸润的轻链型肾淀粉样变性。


关键词: 轻链型淀粉样变性, 多核巨细胞, 肾活检

Abstract: 64-year-old male patient presented as nephrotic syndrome without hypertension and renal function impairment. Blood immunofixation electrophoresis showed λ IgA monoclonal immunoglobulin bands, and blood free light chain examination showed a decreased free κ/λ ratio. Bone marrow flow cytometry showed 4.52% monoclonal plasma cells. He was diagnosed with AL type renal amyloidosis by renal biopsy. The patient achieved clinical VGPR after chemotherapy and repeat renal biopsy showed renal AL amyloidosis with prominent multinuclear giant cell formation.


Key words: light chain amyloidosis, multinucleated giant cells, renal biopsy