Edemigency syndrome as initial presentation of AL form renal amyloidosis: case report
Síndrome edemigência como apresentação inicial de amiloidose renal forma AL: relato de caso
DOI:
https://doi.org/10.53660/CLM-3561-24L16Palavras-chave:
Amyloid, Kidney Diseases, Immunoglobulin Light ChainsResumo
Introduction: Immunoglobulin light chain (AL) amyloidosis is the most common type of amyloidosis, mainly affecting the heart, kidneys and central nervous system. Objective: To report a case of AL-form renal amyloidosis in a patient with initial clinical presentation of edemigenic syndrome. Case report: A 46-year-old man presented with generalized edema and dyspnea. An investigation began into the possible etiologies of edemigenic syndrome, in which renal etiology was the main hypothesis. Protein electrophoresis showed a peak in alpha 2 globulin and serum immunofixation with the presence of a monoclonal lambda band (light chain), then abdominal fat pad biopsy revealed amyloid deposits in the dermis and subcutaneous cellular tissue, confirming the hypothesis of primary amyloidosis with renal involvement. A renal biopsy was performed, which showed amyloid deposits in the glomerular compartment. Bortezomib was started, but there was a progressive worsening of renal function, leading to renal replacement therapy before starting the medication. Conclusion: AL amyloidosis is a progressive disease in which early diagnosis is essential to avoid irreversible losses of organ function.
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