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Kidney Week

Abstract: TH-PO518

LECT2 Amyloidosis in a Pediatric Renal Allograft

Session Information

  • Trainee Case Reports - I
    October 25, 2018 | Location: Exhibit Hall, San Diego Convention Center
    Abstract Time: 10:00 AM - 12:00 PM

Category: Trainee Case Reports

  • 1600 Pediatric Nephrology

Authors

  • Shamir, Eliah, UC San Francisco Medical Center, San Francisco, California, United States
  • Lee, Marsha May, UC San Francisco Medical Center, San Francisco, California, United States
  • Walavalkar, Vighnesh, UC San Francisco Medical Center, San Francisco, California, United States
Introduction

LECT2 amyloidosis is a common form of systemic amyloidosis. We present, to our knowledge, the first case of ALECT2 in a pediatric renal allograft.

Case Description

A 19 y/o Hispanic male with ESRD from posterior urethral valves, s/p living unrelated kidney transplant (2002, age 4), presented with a sCr of 2.4mg/dl (baseline 1.9mg/dl) and 3.6g/day proteinuria. He received daclizumab induction and was maintained on tacrolimus/MMF/sirolimus/prednisone. Past history was notable for episodes of ACR and C4d+AMR treated with thymoglobulin, IVIg, OKT3 and rituximab; de-novo C1q nephropathy; and ultimately transplant-glomerulopathy. Current biopsy showed Type1B ACR and chronic active AMR (C4d+). During low power ultrastructural analysis, unusual electron-lucent material was noticed in interstitial areas. Closer examination revealed disorganized fibrils measuring 8-12nm in diameter. Subsequent Congo Red staining confirmed scant interstitial amyloid deposits. Routine amyloid typing stains were negative, therefore mass spectrometry was performed, confirming ALECT2. Treatment with thymoglobulin, IVIg and rituximab stabilized the sCr at 1.9mg/dl. Follow-up biopsy showed resolution of the ACR. No further treatment changes were made. At last follow-up renal function was stable.

Discussion

ALECT2 is more common in elder Hispanic patients. ALECT2 can occur as a de-novo or recurrent process in renal transplants. Here we hypothesize that chronic inflammation from repeated episodes of rejection coupled with ethnic predilection resulted in ALECT2. Unexplained proteinuria in Hispanic patients and unusual patterns of amyloid distribution are clues to the diagnosis of ALECT. Detailed ultrastructural analysis could help to discover small amounts of amyloid not discernable by light microsopy. Mass spectrometry helps in cases that cannot be classified by stains. Awareness of ALECT2 in young patients is important due to prognostic implications and recurrence in subsequent allografts. There is no treatment for ALECT2.

Congo Red