Sequential bone marrow and combined liver-kidney transplantations for a non-amyloid light chain deposition disease: A unique case report with a complete remission at 6 years

Carolina R D Carmo , Madalina Uzunov , Nadia Arzouk , Frederic Charlotte , Magali Colombat , Pascal Lebray
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Abstract

Light Chain Deposition Disease (LCDD) is a rare monoclonal gammopathy characterized by deposition of light chains along the basement membranes of various organs, leading to progressive dysfunction (1,2). It has histological features that differ from amyloidosis and is most often characterized by the overproduction of kappa light chains (2). The kidney is usually the most affected organ and is responsible for the initial clinical manifestations, whereas liver dysfunction is less common and generally less severe than kidney involvement (1,3). We present a case of LCDD in a patient with renal dysfunction associated with secondary sclerosing cholangitis (SSC) and severe liver and kidney involvement, efficiently treated with autologous stem cell transplantation followed by double liver-kidney transplantation.

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非淀粉样轻链沉积症的骨髓和肝肾联合移植手术:6年后病情完全缓解的独特病例报告
轻链沉积病(LCDD)是一种罕见的单克隆丙种球蛋白病,其特征是轻链沿各种器官的基底膜沉积,导致进行性功能障碍(1,2)。它的组织学特征不同于淀粉样变性,最常见的特征是卡帕轻链的过度产生(2)。肾脏通常是受影响最严重的器官,也是最初临床表现的罪魁祸首,而肝功能障碍则较少见,且通常不如肾脏受累严重(1,3)。我们介绍了一例LCDD患者,该患者肾功能不全,伴有继发性硬化性胆管炎(SSC)和严重的肝肾受累,通过自体干细胞移植和双肝肾移植得到了有效治疗。
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