Diagnosis and treatment of AL amyloidosis in 2022

Authors

  • Victor H. Jimenez-Zepeda, MD

DOI:

https://doi.org/10.58931/cht.2022.118

Abstract

Light chain (AL) amyloidosis is a rare, progressive and typically fatal disease (when advanced) characterized by organ dysfunction secondary to deposition of misfolded fibrils of immunoglobulin light chains that are produced by clonal plasma cells or B cells. Although less than 10% of AL patients qualify for CRAB criteria of symptomatic myeloma (Calcium elevation, Renal dysfunction, Anemia, and Bone disease), the majority of these patients have significant impairment of vital organs, such as the heart, kidney and liver. This implies that the common risk factors used for the assessment of multiple myeloma (MM) are not applicable to AL. AL amyloidosis affects 8–12 individuals per million person-years, and its clinical presentation is variable depending on the extent and number of vital organs affected. The locations of amyloid deposits can vary among patients, thus contributing to the heterogeneity of the clinical manifestations. The heart and kidney, which are the most affected organs, can lead to renal failure, cardiomyopathy, and pericardial and pleural effusions. Initial symptoms at onset are often non-specific (e.g., weight loss, fatigue). Despite advances in the diagnostic tools and treatment options, early mortality rates remain high; the expected one-year mortality is approximately 30%. Unfortunately, by the time the AL diagnosis is made, and treatment is initiated, the disease has often become advanced.

Author Biography

Victor H. Jimenez-Zepeda, MD

Dr. Victor H. Jimenez-Zepeda is a hematologist practicing in Calgary, AB and a member of the Arnie Charbonneau Cancer Research Institute and Tom Baker Cancer Center. He is co-director of the Amyloidosis Program of Calgary. He serves as a member of the steering committee for the Myeloma Canada Research Network Database and is a member of other prestigious international societies such as the International Myeloma Society, International Society of Amyloidosis, European Hematology Association, and the American Society of Hematology among others.

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Published

2022-03-01

How to Cite

1.
Jimenez-Zepeda VH. Diagnosis and treatment of AL amyloidosis in 2022. Can Hematol Today [Internet]. 2022 Mar. 1 [cited 2024 Apr. 15];1(1):29–35. Available from: https://canadianhematologytoday.com/article/view/1-1-4-jimenez-zepeda

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