Light chain systemic amyloidosis manifested as liver failure complicated with fatal spontaneous splenic rupture: A case report

TL;DR

This case report highlights that light chain systemic amyloidosis can present as liver failure with spontaneous splenic rupture, emphasizing the importance of considering amyloidosis in similar unexplained hepatomegaly cases for timely diagnosis and management.

Contribution

It provides a detailed clinical analysis of a rare presentation of light chain amyloidosis causing liver failure and splenic rupture, underscoring diagnostic challenges and the need for biopsy confirmation.

Findings

Amyloidosis can cause liver failure and splenic rupture.

Diagnosis often requires biopsy and may only be confirmed post-mortem.

Early suspicion is crucial for management and prognosis.

Abstract

For a patient with manifestations of nausea, abdominal distension, spontaneous splenic rupture, obvious liver enlargement, low red blood cells and platelets, yellow sclera, and spider angioma, Congo red staining of liver and spleen tissues indicated amyloidosis. After secondary factors were excluded, the patient was finally diagnosed as chronic liver failure, light chain amyloidosis, spontaneous bacterial peritonitis after cystic resection and splenectomy. This case suggests that for patients with chronic liver failure accompanied by spontaneous splenic rupture and hepatomegaly for unknown reasons, the possibility of amyloidosis should be considered after excluding other factors, such as viral liver disease, autoimmune disease, alcoholic liver disease, genetic metabolic liver disease and liver tumor, and etc. Considering the low clinical incidence rate and poor prognosis, relevant diagnosis depends on the biopsy results, and many patients were not confirmed until autopsy after death. Therefore, once amyloidosis is suspected, it is necessary to have communications with relevant patients and their families on the risks for examination, treatment methods and prognosis as soon as possible.