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一例导致急性肝衰竭的轻链沉积病病例及文献综述

A Case of Light Chain Deposition Disease Leading to Acute Liver Failure and Review of Literature.

作者信息

Gandhi Mustafa, Pasha Syed Bilal, Reznicek Emily, Pasha Syed Raheel, Ertugrul Hamza, Araslanova Adel, Yin Feng, Tahan Veysel

机构信息

Department of Internal Medicine, University of Missouri, Columbia, MO 65211, USA.

Division of Gastroenterology and Hepatology, Department of Internal Medicine, University of Missouri, Columbia, MO 65211, USA.

出版信息

Diseases. 2023 Feb 1;11(1):24. doi: 10.3390/diseases11010024.

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)是一种单克隆免疫球蛋白沉积病,其特征是轻链沉积于软组织和内脏,导致系统性器官功能障碍,并伴有潜在的淋巴增殖性疾病。虽然肾脏是受影响最严重的器官,但LCDD也可见心脏和肝脏受累。肝脏表现可从轻度肝损伤到暴发性肝衰竭。在此,我们报告一例83岁患有意义未明的单克隆丙种球蛋白病(MGUS)的女性患者,她因急性肝衰竭进展为循环性休克和多器官衰竭而就诊于我院。经过全面检查,确诊为肝脏LCDD。与血液学和肿瘤学部门联合讨论了化疗方案,但鉴于其预后较差,家属决定采取姑息治疗途径。尽管对于任何急性病症而言,迅速做出诊断都很重要,但这种病症罕见且数据匮乏,使得及时诊断和治疗具有挑战性。现有文献显示,系统性LCDD化疗的成功率各不相同。尽管化疗取得了进展,但LCDD中的肝衰竭预示着预后不佳,由于该病患病率低,进一步的临床试验很困难。在我们的文章中,我们还将回顾此前关于这种疾病的病例报告。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2727/9944111/58d68668b184/diseases-11-00024-g001.jpg

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