PRIMARY SYSTEMIC AMYLOIDOSIS LEADING TO ADVANCED RENAL AND CARDIAC INVOLVEMENT IN A 30-YEAR OLD MAN

The case of a 30-year-old man with primary systemic amyloidosis is rep orted. Three months prior to admission the patient developed fever, ni ght sweats, dyspnea, and bilateral ankle swelling. Recurrent left-side d pleural effusion led to further investigation when massive proteinur ia with free monoclonal lambda chains in the urine became evident. Abd ominal subcutaneous fat aspiration and renal biopsy confirmed the diag nosis of amyloidosis. Bone marrow biopsy and bone scan did not reveal multiple myeloma. Echocardiography showed a sparkling texture of the i nterventricular septum. Pulsed-wave Doppler recording of the left vent ricular inflow profile showed the pattern of advanced cardiac amyloido sis consistent with markedly impaired diastolic heart function. Electr ocardiogram-gated magnetic resonance imaging was carried out for nonin vasive evaluation of cardiac function. The patient was started on repe ated courses of melphalan, prednisone, and colchicine therapy. Despite increasing deterioration of renal function the therapy was tolerated quite well, and the patient is still alive 10 months after initial dia gnosis. Although very rare in this age, primary systemic amyloidosis s hould be considered as a cause of pleural effusion, proteinuria, and c ongestive heart failure and should lead to further investigation.