Renal AL Amyloidosis: Updates on Diagnosis, Staging, and Management

AL amyloidosis is caused by the excessive production of nonfunctional immunoglobulins, leading to the formation of amyloid fibrils that damage vital organs, especially the heart and kidneys. AL amyloidosis presents with non-specific symptoms such as fatigue, weight loss, numbness, pain, and nephroti...

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Bibliographic Details
Published in:Journal of clinical medicine 2024-03, Vol.13 (6), p.1744
Main Authors: Shafqat, Areez, Elmaleh, Hassan, Mushtaq, Ali, Firdous, Zaina, Ashruf, Omer, Mukhopadhyay, Debduti, Ahmad, Maheen, Ahmad, Mahnoor, Raza, Shahzad, Anwer, Faiz
Format: Article
Language:English
Subjects:
Abdomen
Amyloidosis
Biomarkers
Biopsy
Bone marrow
Cardiomyopathy
Care and treatment
Corticosteroids
Development and progression
Diagnosis
Health aspects
Hemodialysis
Immunoglobulins
Immunotherapy
Kidney diseases
Medical prognosis
Microscopy
Patients
Plasma
Review
Scintigraphy
Tomography
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Summary:AL amyloidosis is caused by the excessive production of nonfunctional immunoglobulins, leading to the formation of amyloid fibrils that damage vital organs, especially the heart and kidneys. AL amyloidosis presents with non-specific symptoms such as fatigue, weight loss, numbness, pain, and nephrotic syndrome. Consequently, diagnosis is often delayed, and patients typically present with advanced disease at diagnosis. The Pavia renal staging model stratifies patients based on their likelihood of progressing to dialysis. Treatment with daratumumab plus cyclophosphamide, bortezomib, and dexamethasone (i.e., Dara-CyBorD) was effective in inducing renal response in the landmark phase III ANDROMEDA trial and reducing early mortality. However, determining the most appropriate treatment regimen for relapsed or refractory cases remains a challenge due to various patient- and disease-related factors. Encouragingly, t(11:14) may be a positive indicator of therapy responses to the anti-BCL2 therapy venetoclax. Moreover, it is increasingly possible-for the first time-to clear AL amyloid fibrils from peripheral organs by leveraging novel anti-fibril immunotherapeutic approaches, although these medications are still under investigation in clinical trials. Given these advancements, this review provides a comprehensive overview of the current strategies for diagnosing, staging, treating, and monitoring AL amyloidosis, emphasizing renal involvement.
ISSN:2077-0383
2077-0383
DOI:10.3390/jcm13061744