From the Guidelines
For patients with hypertransaminasemia and a liver biopsy confirming hepatic amyloidosis, management should focus on treating the underlying cause of amyloidosis and alleviating symptoms.
Recommendation
- Discontinue any potentially hepatotoxic medications.
- Consider colchicine 0.6 mg orally twice daily for symptom management, as it may help reduce amyloid deposition 1.
- In cases of primary amyloidosis (AL amyloidosis), consider referral to a hematologist for evaluation of chemotherapy or novel therapies such as daratumumab or bortezomib 1.
- For hereditary amyloidosis (ATTR amyloidosis), consider referral to a specialist for evaluation of tafamidis or patisiran.
- Monitor liver function tests and adjust management as needed to prevent further liver injury.
- Consider supportive care, including management of malnutrition and pruritus, as needed. It is essential to work with a multidisciplinary team, including a hepatologist, hematologist, and other specialists, to develop an individualized treatment plan. Key considerations include:
- ASCT-eligibility for patients with AL amyloidosis, as it can provide a robust and durable response 1.
- The use of bortezomib-based regimens for induction chemotherapy, especially in patients with high disease burden or foreseeable delay in ASCT 1.
- The incorporation of daratumumab in the treatment of AL amyloidosis, particularly in patients who are not eligible for ASCT 1.
From the Research
Management Approach for Hypertransaminasemia with Hepatic Amyloidosis
The management approach for hypertransaminasemia with a liver biopsy confirming hepatic amyloidosis involves:
- Treating the underlying amyloid type with supportive measures to alleviate specific gastrointestinal symptoms 2
- Considering liver transplant, which has been found to have positive outcomes, especially in patients with specific variants of hereditary amyloidosis 2
- Systemic chemotherapy, which may be beneficial for some patients 3
- Addressing the underlying cause of the amyloidosis, such as multiple myeloma, with chemotherapy 4, 5
Diagnostic Considerations
- Liver biopsy is a safe and important diagnostic tool for confirming hepatic amyloidosis 3
- Congo red dye staining with characteristic green birefringence under cross-polarized light is used for histopathological confirmation 2, 5
- Free light chain (FLC) testing can be helpful in identifying monoclonal gammopathies, even when immunoelectrophoretic tests are normal 5