Immunoglobulin Light Chain Amyloidosis with Severe Liver Dysfunction Accompanied by Factor X Deficiency

Area of Science:

• Hematology
• Hepatology
• Pathology

Background:

• Immunoglobulin light chain (AL) amyloidosis is a plasma cell disorder.
• Hepatic involvement in AL amyloidosis is uncommon but can be severe.
• Factor X deficiency can occur in AL amyloidosis, leading to bleeding complications.

Observation:

• A patient with AL amyloidosis presented with a bleeding tendency due to factor X deficiency.
• The patient exhibited marked hepatic involvement by amyloidosis.
• The patient developed severe liver dysfunction and died two weeks after admission.

Findings:

• Autopsy confirmed AL-λ amyloidosis with the liver and spleen as primary sites.
• Severe liver dysfunction was the cause of death.
• Factor X deficiency contributed to the bleeding tendency.

Implications:

• This case underscores that severe hepatic failure can be a fatal complication of AL amyloidosis.
• Early recognition and management of hepatic involvement are crucial for improving patient prognosis.
• Treatment strategies for advanced AL amyloidosis with liver dysfunction need careful consideration due to potential toxicity.