Abstract：
Light chain deposition disease (LCDD) is a monoclonal immunoglobulin deposition disease characterized by light chain deposition in soft tissues and viscera, causing systemic organ dysfunction with an underlying lymphoproliferative disorder. While the kidney is the most affected organ, cardiac and hepatic involvement is also seen with LCDD. Hepatic manifestation can range from mild hepatic injury to fulminant liver failure. Herein, we are presenting a case of an 83-year-old woman with a monoclonal gammopathy of undetermined significance (MGUS), who presented to our institution with acute liver failure progressing to circulatory shock and multiorgan failure. After an extensive workup, a diagnosis of hepatic LCDD was determined. In conjunction with the hematology and oncology department, chemotherapy options were discussed, but given her poor prognosis, the family decided to pursue a palliative route. Though establishing a prompt diagnosis is important for any acute condition, the rarity of this condition, along with paucity of data, makes timely diagnosis and treatment challenging. The available literature shows variable rates of success with chemotherapy for systemic LCDD. Despite chemotherapeutic advances, liver failure in LCDD indicates a dismal prognosis, where further clinical trials are difficult owing to the low prevalence of the condition. In our article, we will also be reviewing previous case reports on this disease.
摘要：
轻链沉积病(LCDD)是一种以软组织和内脏轻链沉积为特征的单克隆免疫球蛋白沉积病,引起全身器官功能障碍并伴有潜在的淋巴增生性疾病。虽然肾脏是受影响最大的器官，心脏和肝脏受累也见于LCDD。肝脏表现可以从轻度肝损伤到暴发性肝衰竭。在这里，我们正在介绍一例83岁的女性，患有不明意义的单克隆丙种球蛋白病（MGUS），他向我们的机构提出了急性肝功能衰竭，进展为循环性休克和多器官衰竭。经过广泛的检查，确定了肝LCDD的诊断。与血液科和肿瘤科合作，讨论了化疗方案，但鉴于她的预后不佳,一家人决定采取姑息治疗的方法。尽管迅速诊断对任何急性疾病都很重要，这种情况的罕见，随着数据的匮乏，及时诊断和治疗具有挑战性。现有文献显示，全身性LCDD的化疗成功率不同。尽管化疗取得了进展，LCDD的肝功能衰竭表明预后不佳，由于该疾病的患病率较低，因此难以进行进一步的临床试验。在我们的文章中,我们还将审查以前关于这种疾病的病例报告。
