Division of Kidney Disease and Hypertension, Department of Medicine, Brown University, Warren Alpert Medical School, Providence, RI, USA.
Nephrol Dial Transplant. 2010 May;25(5):1708-13. doi: 10.1093/ndt/gfp693. Epub 2010 Jan 12.
We report the development of IgA nephropathy (IgAN) following full myeloablative allogeneic hematopoietic cell transplantation in two patients with human leukocyte antigen (HLA) matched sibling donors, unrelated to active or chronic graft-versus-host disease. Both recipients had elevated urinary levels of galactose-deficient IgA1, and one donor-recipient pair had elevated serum levels of galactose-deficient IgA1. We propose that IgAN developed after bone marrow transplantation due to a non-graft-versus-host-disease-related multi-hit process associated with glomerular deposition of galactose-deficient IgA1. These two cases provide unique insight into the kinetics of overproduction of galactose-deficient IgA1 and its glomerular deposition and consequential renal injury in IgAN.
我们报告了 2 例 HLA 匹配的同胞供者全相合异基因造血细胞移植后发生 IgA 肾病（IgAN）的病例，这与活跃或慢性移植物抗宿主病无关。两名受者的尿中均有半乳糖缺乏 IgA1 水平升高，一对供受者的血清中半乳糖缺乏 IgA1 水平升高。我们提出，由于与移植物抗宿主病无关的多打击过程导致半乳糖缺乏 IgA1 在肾小球沉积，骨髓移植后发生了 IgAN。这两例病例为 IgAN 中半乳糖缺乏 IgA1 的过度产生及其在肾小球沉积和随后的肾损伤的动力学提供了独特的见解。
