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.

中文翻译：

---

源自白细胞趋化因子 2 (ALECT 2) 的临床隐匿性淀粉样变性伴心脏受累并发肾移植：病例报告和文献回顾

介绍

源自白细胞趋化因子 2 (ALECT2) 的淀粉样变性可能与缓慢进展的肾功能衰竭有关，在移植时临床上未预料到这种情况。虽然这在临床上通常是微不足道的，但我们报告了一个广泛的全身性 ALECT2 淀粉样变性病例，该病例还涉及心肌，导致肾移植术后围手术期死亡。

案例描述

一名 72 岁西班牙裔女性因继发于高血压的终末期肾病接受肾移植。她入院时心动过缓。移植前的心脏检查未发现浸润过程。移植后低血压性心动过缓骤停会导致多器官衰竭、缺氧性脑损伤和死亡。尸检显示在天然肾脏、肾上腺、脾脏中有大量淀粉样蛋白沉积，肝脏和心肌浸润较少。从小静脉到毛细血管、小动脉和动脉的心脏壁内脉管系统显示淀粉样蛋白沉积。质谱显示 ALECT2 作为淀粉样蛋白。

讨论

ALECT2 是一种系统性淀粉样变性，通常累及肾脏、肾上腺、脾脏和肝脏。在肾移植时临床上可能未预料到它，应在老年患者中考虑，尤其是来自 ALECT2 淀粉样蛋白患病率较高的人群。与全身性疾病相关的并发症可能会增加移植后的发病率或死亡率。ALECT2 淀粉样变性的心脏受累以前并未被确定为重要的临床或尸检发现，但我们的病例表明，在广泛全身性疾病的病例中，心血管系统可能确实很少受 ALECT2 淀粉样变性的影响，并且可能与显着的临床后遗症有关.