AL Amyloidosis

dok_zlo — 16.08.2024

A 58-year-old man presented to the dermatology clinic with a 2-year history of eyelid lesions and with weight loss and fatigue over the past several months. On physical examination, scattered periorbital petechiae and purpura were noted, as well as coalescing, waxy papules on the eyelids (Panel A). Macroglossia was also present. Serum coagulation tests were normal. A biopsy of the left upper eyelid resulted in ecchymosis (Panel B). Histopathological testing of the biopsy specimen showed amorphous extracellular material in the subepithelial and perivascular regions, with positive Congo red staining (Panel C) and apple-green birefringence under polarized light microscopy (Panel D). Direct immunofluorescence of the biopsy specimen also showed predominant lambda light-chain deposition. Serum free light-chain testing, urine protein electrophoresis, and a bone marrow biopsy were subsequently performed. A final diagnosis of AL amyloidosis associated with lambda light-chain myeloma was made. In AL amyloidosis (also called amyloid light-chain or primary amyloidosis), infiltration of vessel walls by amyloid results in vascular fragility. Easy bruising may develop — including in a periorbital “raccoon eyes” pattern — and worsen in response to trauma, as occurred after the biopsy in this patient. Treatment with daratumumab, bortezomib, cyclophosphamide, and dexamethasone was started. After three cycles of therapy, a partial response was observed, and autologous stem-cell transplantation was planned.

Miguel Mansilla Polo, M.D. , and Margarita Llavador-Ros, M.D.Author Info & Affiliations
Published August 14, 2024
N Engl J Med 2024;391:640
DOI: 10.1056/NEJMicm2403455
VOL. 391 NO. 7

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