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Abstract: PO2263

ALECT2 Amyloidosis with Cardiac Involvement Complicating Renal Transplantation

Session Information

Category: Trainee Case Report

  • 1602 Pathology and Lab Medicine: Clinical

Authors

  • Grieff, Anthony N., Rutgers Robert Wood Johnson Medical School, Piscataway, New Jersey, United States
  • Fyfe-Kirschner, Billie S., Rutgers Robert Wood Johnson Medical School, Piscataway, New Jersey, United States
  • Ali, Mahmoud, Rutgers Robert Wood Johnson Medical School, Piscataway, New Jersey, United States
  • Bongu, Advaith, Rutgers Robert Wood Johnson Medical School, Piscataway, New Jersey, United States
  • Puri, Sonika, Rutgers Robert Wood Johnson Medical School, Piscataway, New Jersey, United States
  • Matsuda, Kant Michael, Rutgers Robert Wood Johnson Medical School, Piscataway, New Jersey, United States
Introduction

ALECT2 amyloidosis may be associated with slowly progressive renal failure that is clinically unsuspected at the time of transplantation. While this is typically not clinically significant, we report a case with extensive systemic ALECT2 amyloidosis that also involved the myocardium, contributing to perioperative death post renal transplantation.

Case Description

A 72-year-old Hispanic woman presented for renal transplantation due to ESRD from hypertension. She was bradycardic on admission. Cardiac workup prior to transplantation had not identified an infiltrative process. Post-transplant hypotensive bradycardiac arrests lead to multiorgan failure, anoxic brain injury and death. Autopsy revealed massive amyoid deposition in the native kidneys, adrenals, spleen, and less extensive infiltration of liver and myocardium. Cardiac intramural vasculature from venules to capillaries, arterioles and arteries showed amyloid deposition. Mass spectrometry revealed ALECT2 as the amyloidogenic protein.

Discussion

ALECT2 is a systemc amyloidosis that typically involves kidneys, adrenals, spleen and liver. It may be clinically unsuspected at the time of renal transplantation and should be considered in older patients, especially from higher ALECT2 amyloid prevalence populations.
Complications related to systemic disease may add to morbidity or mortality post-transplantation.
Cardiac involvement in ALECT2 amyloidosis has not been previously identified as a significant clinical or autopsy finding, but our case demonstrates that the cardiovascular system may indeed rarely be involved by ALECT2 amyloidosis and be associated with clinical sequelae.

Diffuse amyloid deposition in native kidneys, adrenals and involving vasculature of the myocardium.