Light Chain (AL) Cardiac Amyloidosis: A Diagnostic Dilemma

Abdelazeem, Basel; Manasrah, Nouraldeen; Yousaf, Amman; Gjeka, Rudin; Kunadi, Arvind

doi:10.7759/cureus.19278

Cited by 2 publications

(3 citation statements)

References 13 publications

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“…Elevated BNP and troponins can be found in those with cardiac involvement. The echocardiogram is the initial diagnostic test of choice, and the first finding is relative apical sparing of longitudinal strain [ 2 ]. Other findings include symmetric or asymmetric ventricular hypertrophy, reduced EF <50%, restrictive diastolic pattern, and pericardial effusion [ 19 ].…”

Section: Discussionmentioning

confidence: 99%

“…Other findings include symmetric or asymmetric ventricular hypertrophy, reduced EF <50%, restrictive diastolic pattern, and pericardial effusion [ 19 ]. Cardiac magnetic resonance imaging is the test of choice for diagnosing cardiac amyloidosis with a characteristic appearance of late gadolinium enhancement across the entire subendocardial circumference [ 2 ]. An endomyocardial biopsy is considered a gold standard for the diagnosis of cardiac amyloidosis.…”

Section: Discussionmentioning

confidence: 99%

“…Amyloidosis has 22 different types of localized forms and 18 types of systemic forms. The most common systemic forms are light chain (AL) amyloidosis and transthyretin amyloidosis [ 2 ]. Light chain (AL) amyloidosis is a rare, heterogeneous condition that affects approximately 8–12 new individuals per million person-years [ 3 ].…”

Section: Introductionmentioning

confidence: 99%

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Do Not Ignore Those Raccoon Eyes; They May Indicate Lethal AL Amyloidosis

2022

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Light chain (AL) amyloidosis is a lethal form of systemic amyloidosis that arises from the clonal expansion of CD38+ plasma cells. Organ damage occurs when these plasma cells produce misfolded immunoglobulin light chains, which form amyloid fibrils and deposit in tissues. A minority of patients with AL amyloidosis show “raccoon eyes” caused by increased vascular fragility from accumulation of amyloid fibrils. Amyloidosis can be directly associated with bleeding diathesis due to factor X deficiency as factor X binds to amyloid fibrils primarily in the liver and spleen. A 65-year-old Caucasian male presented with random bruising in the upper chest and around the eyes for 1.5 years. Physical examination was unremarkable, except for neck bruising. Pertinent workup showed protein electrophoresis with a faint M spike, increased serum lambda light chains, a kappa to lambda ratio of 0.06, increased Bence-Jones proteins, reduced factor X activity, elevated NT-proBNP. The bone marrow biopsy was positive for Congo red stain for amyloid protein. Magnetic resonance imaging revealed diffuse enhancement of the right and left ventricle subendocardial late gadolinium, consistent with cardiac amyloidosis. The patient started systemic therapy with a regimen of daratumumab, cyclophosphamide, bortezomib, and dexamethasone. After one cycle of therapy, lambda light chains normalized with an improvement in bruising. Diagnostic delays for cardiac patients are concerning as the median survival rate among these patients, when not treated, is approximately 6 months after the onset of symptoms. Since timely treatment can prevent organ damage, clinicians should be aware of specific clinical signs such as raccoon eyes and the importance of systemic evaluation for a prompt diagnosis.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Do Not Ignore Those Raccoon Eyes; They May Indicate Lethal AL Amyloidosis

2022

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Associations of Patients with Pericardial Effusion Secondary to Light-Chain or Transthyretin Amyloidosis- A Systematic Review

Javed,

Singh,

Shirah

et al. 2024

CCR

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Background:: Pericardial effusion is associated with amyloidosis, specifically amyloid light chain (AL) and transthyretin (ATTR) subtypes. However, the patients might present with different clinical symptoms. Objective:: To determine the characteristics and associations of patients with pericardial effusion owing to either AL or ATTR amyloidosis. Methods:: This study reviewed 26 studies from databases such as PubMed, MEDLINE, Web of Science, Google Scholar and CINAHL databases after protocol registration. The data were analyzed in IBM SPSS 21. Many statistical tests, such as Student t- and the Mann-Whitney U tests, were used. Multivariate logistic regression analysis was also performed. A p-value< 0.05 was considered significant. Results:: A total of 531 patients with pericardial effusion secondary to amyloidosis were included. The mean age was 58.4±24.5 years. Most of the patients were male (72.9%). Common co-morbid conditions included hypertension (16.8%) and active smoking (12.9%). The most common time from symptom onset to the clinical presentation was less than 1 week (45%). ATTR amyloidosis was more common in older patients (p<0.05). Abdominal and chest discomfort were commonly associated with AL and ATTR amyloidosis, respectively (p<0.05). Patients with AL amyloidosis had a higher association with interventricular septal thickening and increased posterior wall thickness (p<0.05). First-degree atrioventricular block, left bundle branch block (LBBB), and atrial fibrillation (AF) were more associated with ATTR amyloidosis (p<0.05). Conclusion:: Pericardial effusion in patients with AL amyloidosis was associated with hypertrophic remodeling, while conduction abnormalities were associated with ATTR amyloidosis.

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Light Chain (AL) Cardiac Amyloidosis: A Diagnostic Dilemma

Cited by 2 publications

References 13 publications

Do Not Ignore Those Raccoon Eyes; They May Indicate Lethal AL Amyloidosis

Do Not Ignore Those Raccoon Eyes; They May Indicate Lethal AL Amyloidosis

Associations of Patients with Pericardial Effusion Secondary to Light-Chain or Transthyretin Amyloidosis- A Systematic Review

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