RESUMO
Background: Drug-induced myocarditis is a rare complication of certain cancer treatments, characterized by the development of myocardial inflammation shortly after initiation of treatment, potentially leading to heart failure and/or malignant arrhythmias. The development of eosinophilic myocarditis after administration of lenalidomide has been described and bortezomib has been associated with the development of cardiomyopathies and atherosclerosis. Case summary: A 69-year-old woman, recently diagnosed with multiple myeloma underwent local radiotherapy for a pathological fracture of the 4th lumbar vertebra and was treated with bortezomib-lenalidomide-dexamethasone. Within 19 days after therapy initiation, she presented with gastrointestinal symptoms, an erythematous pruritic rash, and general fatigue. Surprisingly, routine electrocardiogram (ECG) showed upwardly concave ST-elevation in I and aVL and ST-depressions in II, III, and aVF. Troponin levels were markedly elevated to 5470 ng/L. Complete blood count revealed eosinophilia. Based on further cardiac work-up, including echocardiography, coronary angiography, and cardiac magnetic resonance imaging (MRI) showing positive T2 imaging and patchy subepicardial late gadolinium enhancement, she was diagnosed with hypersensitivity myocarditis. Additional endomyocardial heart biopsy did not reveal any abnormalities, probably due to sampling error. After discontinuation of chemotherapy and prompt treatment with high doses of corticosteroids, the patient recovered. Discussion: Diagnosis of drug-induced myocarditis can be challenging and even long known widely used (chemo)therapy should be considered a potential trigger. Early diagnosis and treatment are crucial, warranting alertness for suggestive symptoms. Cardiac biomarkers, ECG monitoring, and cardiac MRI are key to confirm the diagnosis. In patients with preserved left ventricular systolic function, two-dimensional speckle tracking echocardiography can provide additional diagnostic information. Every patient presenting with eosinophilia and/or acute onset of auto-immune symptoms after initiation of therapy with lenalidomide/bortezomib deserves prompt cardiac screening. The gold standard remains an endomyocardial biopsy, although sampling error may occur.
RESUMO
Background: Light-chain amyloidosis has always been described as a sporadic disease caused by plasma cell dyscrasia. Cardiac amyloidosis refers to cardiac involvement with infiltration of amyloid fibrils in the myocardium. The degree of cardiac involvement is the greatest predictor of prognosis. To our knowledge, AL cardiac amyloidosis has only been reported once before in first-degree relatives. Case summary: In this report, we describe the unusual cases of two sisters with light-chain cardiac amyloidosis. The first patient underwent autologous stem cell transplantation and remained in remission for 10 years until the disease relapsed and she died of end-stage heart failure. The second patient was promptly started on a chemotherapy regimen but died shortly after her initial diagnosis due to rapid progression of cardiac dysfunction. Conclusion: Cardiac amyloidosis is a severe life-threatening condition which requires a multidisciplinary diagnostic and therapeutic approach. Based on this case report, a genetic cause for AL amyloidosis might be suspected or is this a purely coincidental finding? Counselling, screening, and follow-up of other family members are very challenging. As is often the case with rare diseases, many unsolved questions remain, representing important challenges for clinicians.