Sudden death in lambda light chain AL cardiac amyloidosis: a review of literature and update for clinicians and pathologists.
International journal of clinical and experimental pathology, ISSN: 1936-2625, Vol: 13, Issue: 7, Page: 1474-1482
2020
- 6Citations
- 21Captures
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Example: if you select the 1-year option for an article published in 2019 and a metric category shows 90%, that means that the article or review is performing better than 90% of the other articles/reviews published in that journal in 2019. If you select the 3-year option for the same article published in 2019 and the metric category shows 90%, that means that the article or review is performing better than 90% of the other articles/reviews published in that journal in 2019, 2018 and 2017.
Citation Benchmarking is provided by Scopus and SciVal and is different from the metrics context provided by PlumX Metrics.
Metrics Details
- Citations6
- Citation Indexes6
- Captures21
- Readers21
- 21
Review Description
Light chain (AL) amyloidosis is the most common type of systemic amyloidosis, affecting around 10 people per million per year. In Europe, approximately 5000 new diagnosis per year are reported. Deposition of amyloid fibrils derived from antibody light chains are key pathogenic agents in AL amyloidosis. They can be deposited in multiple organs but cardiac involvement carries a major risk of mortality. The prognosis is poor in cases associated with multiple myeloma. The average survival is around 1 year. Up to half of all patients with cardiac amyloidosis die suddenly; 75% ofthose deaths are due to heart failure. Ventricular arrhythmia is also associated with cardiac amyloidosis and unexpected death. It is crucial to make a diagnosis and start treatment at an early stage. Recent data suggest that cardiac amyloidosis has become a treatable and curable condition with a combination of agents targeting multiple steps of the amyloid cascade. ICD implantation may not be as effective for the therapy of light chain (AL) cardiac amyloidosis as supposed earlier. In cases of unexpected and sudden death, autopsy may show unknown conditions and is valuable to assess existing risks for family members. Even after careful autopsy, a proportion of sudden deaths, ranging from 2 to 54%, remain unexplained and this broad range of values is likely due to the heterogeneity of autopsy protocols. Post mortem diagnosis of cardiac amyloidosis still represents a challenge for forensic pathologists. Detailed morphologic study of the heart and a complete histopathologic study are mandatory. Immunohistochemistry is essential for amyloid subclassification. A review of existing literature is performed by the authors and a methodological approach in post mortem diagnosis of light chain AL cardiac amyloidosis is proposed. Both macroscopic and microscopic findings are discussed.
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