Bad players in AL amyloidosis in the current era of treatment
Natalia Kreiniz1,2, Morie A Gertz3
1Division of Hematology, Bnai Zion Medical Centre, Haifa, Israel.
Insights
Systemic AL amyloidosis (ALA) prognosis is improving with new therapies. Advanced cardiac imaging and novel biomarkers offer promising, more specific assessments for this plasma cell disorder.
Area of Science:
- Hematology
- Oncology
- Cardiology
Background:
- Systemic AL amyloidosis (ALA) is a plasma cell disorder with poor prognosis, historically linked to cardiac involvement.
- Current prognostic models rely on cardiac markers and dFLC, but cardiac biomarkers lack specificity and are influenced by other factors.
- Recent therapeutic advances have significantly improved patient outcomes in ALA.
Purpose of the Study:
- To provide an updated review on the prognostic significance of cardiac involvement in ALA.
- To discuss the role of novel diagnostic and prognostic markers, including imaging and biological markers.
- To highlight the impact of modern therapies and minimal residual disease (MRD) assessment on ALA prognosis.
Main Methods:
- Literature search conducted in PubMed and Google Scholar.
- Inclusion of recent and relevant data on ALA prognosis, treatment response, and biomarkers.
- Focus on advancements in cardiac imaging (ECHO, MRI) and biological markers.
Main Results:
- Cardiac involvement remains a key prognostic factor in ALA.
- Newer imaging modalities like ECHO and MRI provide more precise cardiac assessment.
- Emerging biological markers related to cardiac injury, inflammation, and endothelial damage show promise.
- Minimal residual disease (MRD) assessment is crucial for evaluating treatment response.
Conclusions:
- Modern therapies have transformed ALA prognosis.
- Advanced cardiac imaging and novel biomarkers offer improved risk stratification.
- Prospective multicenter trials are needed to refine risk assessment models in the current therapeutic landscape.
Introduction:
Systemic AL amyloidosis (ALA) is a clonal plasma cell (PC) disease characterized by deposition of amyloid fibrils in different organs and tissues. Traditionally, the prognosis of ALA is poor and is primarily defined by cardiac involvement. The modern prognostic models are based on cardiac markers and free light chain difference (dFLC). Cardiac biomarkers have low specificity and are dependent on renal function, volume status, and cardiac diseases other than ALA. New therapies significantly improved the prognosis of the disease. The advancements in technologies - cardiac echocardiography (ECHO) and cardiac MRI (CMR), as well as new biological markers, relying on cardiac injury, inflammation, endothelial damage, and clonal and non-clonal PC markers are promising.
Areas Covered:
An update on the prognostic significance of cardiac ALA, number of involved organs, response to treatment, including minimal residual disease (MRD), ECHO, MRI, and new biological markers will be discussed. The literature search was done in PubMed and Google Scholar, and the most recent and relevant data are included.
Expert Opinion:
Prospective multicenter trials, evaluating multiple clinical and laboratory parameters, should be done to improve the risk assessment models in ALA in the modern era of therapy.
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