Korean J Transplant.  2020 Mar;34(1):66-70. 10.4285/kjt.2020.34.1.66.

Amyloid light-chain amyloidosis presented as focal segmental glomerulosclerosis in a kidney transplant recipient

Affiliations
  • 1Transplant Research Center, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
  • 2Division of Nephrology, Department of Internal Medicine, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
  • 3Department of Hospital Pathology, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
  • 4Convergent Research Consortium for Immunologic Disease, Seoul St. Mary’s Hospital, The Collage of Medicine, The Catholic University of Korea, Seoul, Korea

Abstract

Amyloid light-chain (AL) amyloidosis after kidney transplantation is a rare disease in the world, and only one case of systemic AL amyloidosis after kidney transplantation is reported in Korea. We here report a 46-year-old woman with AL amyloidosis developed after kidney transplantation. The underlying disease of our case was focal segmental glomerulosclerosis (FSGS), and was admitted to hospital for evaluation of proteinuria developed 2 years after kidney transplantation. The patient was initially diagnosed as recurrent FSGS on light microscopy. But, electron microscopic finding was suggestive of amyloidosis and systemic evaluation was consistent with systemic AL amyloidosis. This case provides the importance of differential diagnosis of proteinuria in kidney transplant recipients.

Keyword

Amyloid light-chain amyloidosis; Kidney transplantation; Focal segmental glomerulosclerosis

Figure

  • Fig. 1 Urine protein electrophoresis shows heavy albuminuria but no monoclonal protein. alb, albumin.

  • Fig. 2 H&E stained section revealed small amounts of amorphous eosinophilic material deposition (arrows), which were Congo-red positive (inlet) in the glomerular mesangium (×400).

  • Fig. 3 Immunofluorescence staining revealed small amounts of mesangial deposition of lambda light chains (×400).

  • Fig. 4 Electron microscopy showed small amounts of haphazardly arranged non-branching fine fibrils, about 8–10 nm in thickness in the mesangial areas.


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