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Raccoon eyes in systemic light chain amyloidosis

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A 51-year-old man presented with a 3-year history of recurrent upper eyelid haematomas. He had a low level IgA lambda paraprotein in blood, Bence–Jones protein and a lambda predominance on serum free light chain ratio. Biopsies of his eyelids showed vascular infiltration by amyloid fibrils (lambda light chains) establishing a diagnosis of light chain (AL) amyloidosis. The patient had cutaneous involvement only at diagnosis. He underwent an autologous stem cell transplant 4 years after the initial presentation, at which time there was evidence of early cardiac involvement by AL amyloidosis manifest by increased B-type natriuretic peptide and confirmed on cardiac magnetic resonance imaging. While thrombocytopenic during his transplant, the patient developed bilateral periorbital haematomas, which resolved with platelet engraftment.

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