Related Experiment Video

Updated: May 15, 2026

Genetic Analysis of Hereditary Transthyretin Ala97Ser Related Amyloidosis
06:33

Genetic Analysis of Hereditary Transthyretin Ala97Ser Related Amyloidosis

Published on: June 9, 2018

[Fatal hepatic failure due to AL amyloidosis in a patient with multiple myeloma]

Sumie Tabata1, Masayuki Kurata, June Takeda

  • 1Department of Hematology and Clinical Immunology, Kobe City Medical Center General Hospital, Kobe, Japan.

[Rinsho Ketsueki] the Japanese Journal of Clinical Hematology
|December 22, 2012
PubMed

Insights

A rare case of fatal liver amyloidosis occurred in a multiple myeloma patient. This highlights the importance of monitoring liver function in multiple myeloma patients with AL amyloidosis.

Area of Science:

  • Hematology
  • Oncology
  • Pathology

Background:

  • Multiple myeloma (MM) affects approximately 10-15% of patients with AL amyloidosis.
  • Liver-restricted fatal amyloidosis is an uncommon manifestation of AL amyloidosis.

Observation:

  • A 73-year-old female diagnosed with IgG-κ MM (Stage I) experienced disease progression to Stage III.
  • The patient underwent VAD and CM chemotherapy with limited response.
  • She developed jaundice, elevated biliary enzymes, and later, bleeding tendency, edema, and liver dysfunction.

Findings:

  • Despite chemotherapy, the patient progressed to hepatic coma and died from intracranial hemorrhage.
  • Autopsy revealed that the liver was almost entirely replaced by AL amyloid substance.
  • This case underscores the potential for aggressive, fatal liver involvement in MM-associated AL amyloidosis.

Implications:

  • This case highlights a rare but fatal complication of multiple myeloma and AL amyloidosis.
  • It emphasizes the need for vigilant monitoring of liver function in patients with MM and AL amyloidosis.
  • Early recognition and management of liver involvement may be crucial for improving outcomes.

More Related Videos

Generation of a Rat Model of Acute Liver Failure by Combining 70% Partial Hepatectomy and Acetaminophen09:44

Generation of a Rat Model of Acute Liver Failure by Combining 70% Partial Hepatectomy and Acetaminophen

Published on: November 27, 2019

Related Concept Videos

Amyloid Fibrils03:03

Amyloid Fibrils

Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining, normally used to...
Amyloid Fibrils03:03

Amyloid Fibrils

Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining, normally used to...
Hepatic Encephalopathy01:29

Hepatic Encephalopathy

DefinitionHepatic encephalopathy is a reversible neurologic syndrome that results from advanced liver dysfunction or portosystemic shunting. It leads to disturbances in cognition, behavior, and motor function due to the brain’s exposure to gut-derived toxins that the liver fails to detoxify.EtiologyThis condition develops either in the setting of acute fulminant hepatitis or progressively during chronic liver disease, such as cirrhosis and portal hypertension. Portosystemic shunting—including...
Effect of Hepatic Disease on Pharmacokinetics: Pathophysiologic Assessment and Liver Function Test01:22

Effect of Hepatic Disease on Pharmacokinetics: Pathophysiologic Assessment and Liver Function Test

In clinical practice, the direct measurement of hepatic blood flow to evaluate liver function presents significant challenges due to the intricate and specialized nature of the necessary techniques. Consequently, healthcare professionals often rely on empirical estimates derived from thorough patient examinations and liver function tests to gauge liver health. Among the tools at their disposal, the Child–Pugh and MELD scoring systems stand out for their ability to categorize and assess the...
Cirrhosis II: Pathophysiology01:24

Cirrhosis II: Pathophysiology

Cirrhosis is a progressive chronic liver injury caused by prolonged inflammation, excessive fibrotic remodeling, and impaired regeneration. Over time, repeated hepatic insults disrupt the liver’s architecture and function, leading to reduced blood flow, impaired bile drainage, and diminished metabolic capacity.Pathophysiology of cirrhosisCirrhosis arises from three main responses to chronic liver damage: inflammation, immune activation, and hepatocyte death. These processes lead to structural...
Effect of Hepatic Disease on Pharmacokinetics: Drug Dosing and Hepatic Blood Flow01:26

Effect of Hepatic Disease on Pharmacokinetics: Drug Dosing and Hepatic Blood Flow

Chronic liver disease significantly impacts drug metabolism due to alterations in hepatic blood flow and enzyme accessibility. This disruption affects the body's pharmacokinetics—the movement and processing of drugs within the system. Key enzymes crucial for metabolizing medications become less accessible, changing how drugs are processed and utilized. Furthermore, liver disease influences the synthesis of plasma proteins, such as albumin and globulins, which play critical roles in drug binding...