Laryngeal amyloidosis: Localized versus systemic disease and update on diagnosis and therapy

The clinical and pathological characteristics, possibility of systemic disease, and effect of local therapy were studied in laryngeal amyloidosis. Records of all patients with localized laryngeal amyloidosis in a single tertiary referral center were examined retrospectively at diagnosis and after local therapy. Of 188 new patients with amyloidosis between 1990 and 2003, 5 patients had localized laryngeal amyloidosis. A sixth patient with localized laryngeal amyloidosis turned out to have systemic AL (immunocyte-derived) amyloidosis 8 years later. Free light chains were found in this patient, as well as in 1 of the other 5 patients. Amyloid interfering with laryngeal or airway function was removed during microlaryngoscopy with a carbon dioxide laser or cold endoscopic excision. The best results were seen when glottic deposits were removed by cold endoscopic excision, and supraglottic deposits by a carbon dioxide laser. Four patients had recurrent disease. A systematic workup, including measurement of free light chains, helps to rule out systemic disease.