Primary amyloidoses sistêmica al: story of case and considerações associates
Keywords:
Amiloidose Sistêmica Tipo AL, Amiloidose Sistêmica Primária, Terapêutica.Abstract
Amyloidosis is a condition inherent to a group of diseases, which exhibit the common feature of pathological extracellular deposition of insoluble proteins in organs or tissues. All amyloid fibrils share the same secondary structure, the -pleated sheet conformation, and a nonfibrillar identical component, the serum amyloid pentraxin (SAP). We report a 62-year-old man with systemic AL amyloidosis. Diagnosis was made through histopathology and imaging. The patient underwent 6 cycles of vincristine, adriamycin and dexamethasone (VAD), with maintenance with cyclophosphamide 600mg for 1 day and dexamethasone 40mg/day for 4 days every 28 days. The patient was relieved of his symptoms and returned to his daily activities.Key-words: Systemic AL amyloidosis, Primary Systemic Amyloidosis, Therapeutics.
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