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轻链沉积病(LCDD)是一种全身性疾病。其特征是各器官有能与单型轻链抗血清起反应,而无淀粉样蛋白纤丝超微结构的刚果红阴性物质沉积。主要侵犯肾脏,偶亦有侵犯肝脏的报道,但罕有以肝脏侵犯为主要临床表现者。本文首次报道表现为严重胆汁郁积而无严重肾脏损害的患者。病例报告:患者男性,67岁,因进行性无痛性黄疸三个月入院。体检:黄疸显著,锁骨上、腋下及腹股沟淋巴结轻度肿大,肝脏肋缘下7cm,脾未触及。实验室检查:有明显胆汁郁积;肾功能正常。CT示肝门、主动脉与腔静脉间、肝胃韧带和纵隔淋巴结轻度肿大。
Light chain deposition disease (LCDD) is a systemic disease. It is characterized by the deposition of Congo red-negative material that reacts with the monotype light chain antiserum and without the amyloid fibrillar ultrastructure. The main violations of the kidney, even have also reported violations of the liver, but rare with liver invasion as the main clinical manifestations. This article reports for the first time in patients presenting with severe cholestasis without severe kidney damage. Case Report: Male, 67 years old, admitted to hospital for three months due to progressive painless jaundice. Physical examination: jaundice significant, supraclavicular, underarm and inguinal lymph nodes slightly enlarged liver rib margin 7cm, spleen not touched. Laboratory tests: a clear cholestasis; normal renal function. CT showed hepatic portal, aorta and vena cava, liver and stomach lymph nodes and mediastinal lymph nodes slightly enlarged.