Clinically occult amyloidosis derived from leukocyte chemotactic factor 2 (ALECT 2) with cardiac involvement complicating renal transplantation: case report and literature review

Introduction: Amyloidosis derived from leukocyte chemotactic factor 2 (ALECT2) may be associated with slowly progressive renal failure that is clinically unsuspected at the time of transplantation. While this is typically clinically insignificant, 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 end-stage renal disease secondary to hypertension. She was bradycardic on admission. Cardiac workup prior to transplantation had not identified an infiltrative process. Post-transplant hypotensive bradycardic arrests lead to multiorgan failure, anoxic brain injury, and death. Autopsy revealed massive amyloid 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 systemic 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 in cases with extensive systemic disease, and it may be associated with significant clinical sequelae.