Abstract：
Immunotactoid deposition is a rare fibrillary deposition disease that is primarily seen in the kidney and is associated with paraproteinemia. Here, we report a case of hepatic immunotactoid deposition in a 67-year-old male with a history of smoldering myeloma and chronic kidney disease who underwent liver transplantation for metabolic dysfunction-related cirrhosis. Immunotactoid deposition was first identified in the explanted liver and recurred in the allograft within only 7 weeks following transplantation, presenting as ascites with normal liver function tests. The patient\'s posttransplant course was complicated by proteinuria and renal failure requiring dialysis. Histologic examination of both native and allograft livers demonstrated pink amorphous material occupying sinusoidal spaces that were Congo-red negative and immunoglobulin M Kappa-restricted. Electron microscopy revealed characteristic deposits of electron-dense bundles of hollow microtubules with a 40 nm diameter within the sinusoids and space of Disse, consistent with immunotactoids. Therapy of the patient\'s underlying plasma-cell dyscrasia utilizing a daratumumab-based regimen showed decreased serum paraproteins, resolution of ascites, and improved kidney function, no longer requiring dialysis, without inducing rejection. The patient continues to respond to treatment 10 months posttransplant.
摘要：
类免疫沉积是一种罕见的纤维沉积疾病，主要见于与副蛋白血症相关的肾脏。这里,我们报道1例67岁男性因代谢功能障碍相关性肝硬化而接受肝移植治疗,该患者有多发性骨髓瘤和慢性肾病病史,出现肝免疫类毒素沉积.首先在移植后的肝脏中发现了类免疫沉积，并在移植后仅7周内在同种异体移植物中复发。表现为腹水，肝功能正常。患者的移植后过程并发蛋白尿和肾衰竭，需要透析。天然和同种异体肝脏的组织学检查均显示粉红色无定形物质占据了刚果红阴性且IgMκ限制的正弦空间。电子显微镜显示，在Disse的正弦曲线和空间内，直径为40nm的中空微管的电子致密束的特征沉积物，与免疫类一致。使用基于Daratumumab的方案治疗患者潜在的浆细胞发育不良显示血清副蛋白降低，腹水的分辨率，改善肾功能不再需要透析，不会引起排斥。患者在移植后10个月继续对治疗有反应。
