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[AL amyloidosis--a hematologic disease with initial cardiac manifestation. 2 case reports]
R Berent1, J Auer, C Punzengruber
1II. Interne Abteilung/Kardiologie, Allg. Krankenhaus der Barmh. Schwestern, vom Hl. Kreuz, Wels.
Insights
Cardiac amyloidosis, often linked to multiple myeloma, can cause dyspnea. Suspicious cardiac findings warrant further tests like immunofixation, even with normal labs, to diagnose light chain amyloidosis.
Area of Science:
- Cardiology
- Hematology
- Oncology
Background:
- Cardiac involvement is common in AL-amyloidosis, a complication of multiple myeloma.
- Early diagnosis is crucial for managing this infiltrative cardiomyopathy.
Observation:
- Two patients presented with dyspnea and suspicious cardiac findings.
- Routine lab tests, including ESR and electrophoresis, were normal.
- Echocardiography revealed left ventricular hypertrophy and restrictive mitral flow; ECG showed low voltage and absent R waves.
Findings:
- Hematologic workup confirmed light chain production, indicative of myeloma.
- Infiltrative cardiomyopathy was suspected based on cardiac imaging and ECG findings.
- Immunofixation testing confirmed light chain amyloidosis despite normal initial laboratory results.
Implications:
- Echocardiography and ECG findings suggestive of infiltrative cardiomyopathy warrant further investigation for light chain amyloidosis.
- Immunofixation is essential for diagnosing light chain disease in patients with suspected cardiac amyloidosis, even with normal routine laboratory tests.
- This highlights the importance of a multidisciplinary approach in diagnosing complex hematologic and cardiac conditions.
Abstract:
Cardiac involvement in AL-amyloidosis due to a multiple myeloma is present in up to 90% of cases. We present two patients with cardiac symptoms in whom a hematologic disease could be diagnosed because of suspicious cardiac finding. The leading symptom was dyspnea. The routinely performed laboratory tests, especially the erythrocyte sedimentation rate and the electrophoresis, were normal. After exclusion of coronary artery disease an infiltrative cardiomyopathy was suspected because of the echocardiographic examination with marked left ventricular hypertrophy, the restrictive flow pattern at the mitral valve and the electrocardiogram with a low voltage in limb leads and absent R waves in left precordial leads. Further, hematologic workup confirmed the production of light chains due to a myeloma. If the echocardiographic examination and the electrocardiogram raises the suspicion of an infiltrative cardiomyopathy as the cause of dyspnea, an immunofixation should be done in spite of normal laboratory tests to confirm or rule out the presence of a light chain disease due to a myeloma.