Abstract

BACKGROUND: Amyloidosis is a severe systemic disorder produces by the accumulation of inappropriately amyloid deposition in tissues. Cardiac involvement, as a main type of amyloidosis, has a major impact on prognosis. We describe a biopsy-proven cardiac amyloidosis in an old man with unexpected presentation. CASE REPORT: A 70-year-old man, with a complaint of severe weakness, lightheadedness, and lower limb paresthesia, was admitted to the emergency department. Electrocardiography revealed right bundle branch block and Trifascicular block. Echocardiography study showed a moderately increased thickness of left ventricular wall with concentric pattern as well. Laboratory investigations including serum and urine electrophoresis, and serum free light chain examination as immunofixation assay revealed that kappa chains predominated over lambda chains in a ratio of 3:2. Our patient with final diagnosis of amyloid light-chain (AL) amyloidosis underwent chemotherapy with melphalan combined with high-dose dexamethasone, CPHPC and monoclonal antibodies for 2 weeks. CONCLUSION: It shows that rapid diagnosis of AL amyloidosis can enhance the prognosis. Applying an optimal strategy for the treatment leads to effective therapy, too.