Volume 206 - Issue 9

Raccoon eyes in systemic light chain amyloidosis

Authors:  Cecily J Forsyth and Campbell R Tiley

Med J Aust 2017; 206 (9): 384. || doi: 10.5694/mja16.00848
Published online: 15 May 2017
A 51-year-old man presented with a 3-year history of recurrent upper eyelid haematomas

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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