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Case Reports
. 2024 Apr;54(4):221-222.
doi: 10.4070/kcj.2024.0022.

Cardiomyopathy Without Amyloid Deposit in Systemic Light Chain Deposition Disease

Affiliations
Case Reports

Cardiomyopathy Without Amyloid Deposit in Systemic Light Chain Deposition Disease

Bo Eun Park et al. Korean Circ J. 2024 Apr.
No abstract available

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Conflict of interest statement

The authors have no financial conflicts of interest.

Figures

Figure 1
Figure 1. Images and histological analyses in a case with cardiac light chain deposition disease. (A) Transthoracic echocardiographic image showing left ventricular wall thickness with disproportionate interventricular septal enlargement, resulting in a ‘speckled’ appearance and small pericardial effusion (parasternal long-axis view). (B) Regional longitudinal strain, assessed by 2-dimensional speckle-tracking echocardiography. The reduction in global longitudinal strain is particularly observed in the basal and mid-ventricular segments, manifesting as an ‘apical sparing’ pattern. The mean global longitudinal strain was −8.5%. (C) Electrocardiogram; The electrocardiogram of the patient reveals characteristic findings, including low voltage in the limb leads (QRS amplitude <5 mm in leads II, III, aVR, aVF) and a pseudo-infarct pattern (pathologic Q waves in V1, V2, V3, accompanied by the loss of R wave progression). (D, G) Histological analyses of endomyocardial biopsy specimens. (D) Trichrome staining: The blue areas signify significant interstitial fibrosis, original magnification ×100. (E) Congo red staining; The Congo red stain did not show extracellular deposition of amyloid fibrils in the myocardial interstitium, original magnification ×100. (F) Immunofluorescence using anti-κ light chain antibody; the myocardial interstitium shows a negative reaction, original magnification ×100. (G) Immunofluorescence using anti-λ light chain antibody; The immunostaining with anti-λ light chain antibodies reveals intense positivity in the myocardial interstitium, original magnification ×100.
aVR = augmented vector right; aVF = augmented vector foot.

References

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