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Case Reports
. 2025 Jun 27;6(2):104-109.
doi: 10.47487/apcyccv.v6i2.477. eCollection 2025 Apr-May.

Monoclonal gammopathy progressing to systemic amyloidosis with cardiac involvement. A case report

Germán Valenzuela-Rodríguez  1   2 , Ofelia Araoz Tarco  1   3 , Iván Fernández Vertiz  1   4
Affiliations
Case Reports

Monoclonal gammopathy progressing to systemic amyloidosis with cardiac involvement. A case report

Germán Valenzuela-Rodríguez et al. Arch Peru Cardiol Cir Cardiovasc. .

Abstract
in English, Spanish

We report a case of systemic light chain amyloidosis with cardiac involvement, preceded by a monoclonal IgG lambda gammopathy. The clinical diagnosis was based on signs of heart failure, elevated cardiac biomarkers, and characteristic imaging findings. The diagnosis was confirmed by increased levels of free light chains in blood and urine, as well as the presence of amyloid deposits in periumbilical fat and multiple segments of the gastrointestinal tract. Treatment with daratumumab, bortezomib, and dexamethasone was initiated, followed by autologous hematopoietic stem cell transplantation 22 months after diagnosis, resulting in a favourable clinical outcome.

Reportamos el caso de una amiloidosis sistémica de cadenas ligeras con compromiso cardíaco, precedida de una gammapatía monoclonal por IgG lambda. El diagnóstico clínico se realizó por signos de insuficiencia cardíaca, elevación de biomarcadores y hallazgos de imágenes, orientándose con la elevación de cadenas ligeras en sangre y orina, y confirmándose con la presencia de depósitos de amiloide en la biopsia de grasa periumbilical y en diferentes segmentos del tracto digestivo. Se inició tratamiento con daratumumab más bortezomib más dexametasona, recibiendo un transplante de células madre hematopoyéticas de tipo autólogo a los 22 meses del diagnóstico, con una evolución clínica favorable.

Keywords: Cardiac; Light Chain Amyloidosis; Monoclonal Gammopathy of Undetermined Significance.

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Conflict of interest statement

Conflicts of interest: None

Figures

Figure 1
Figure 1. Twelve-lead electrocardiogram showing low-voltage QRS complexes, incomplete right bundle branch block, and overall reduced voltage.
Figure 2
Figure 2. Echocardiogram demonstrating reduced global longitudinal strain (-12.4) at the time of diagnosis
Figure 3
Figure 3. Cardiac magnetic resonance imaging in T1 inversion recovery (IR) sequences, showing late gadolinium enhancement in three-chamber, four-chamber, and short-axis views. Intramyocardial (focal) fibrosis is observed, predominantly in the interventricular septum and inferior wall (red arrows), with a distribution that is atypical for cardiac amyloidosis.
Figure 4
Figure 4. Area of aberrant plasma cells identified by bone marrow flow cytometry, expressing clonality with the following phenotype: cIgG Lambda ++ / cIgG Kappa - / CD38+ / CD56 +++ / CD20 - / CD117 ++.
Figure 5
Figure 5. Echocardiogram showing improvement in global longitudinal strain two years after diagnosis.
See this image and copyright information in PMC

References

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