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Keywords: Localized amyloidosis, immunolabeling, prognosis
Abbreviations: TTR = transthyretin
Abstract
Since the prognosis of localized amyloidosis remains unclear, we conducted a survey to define the characteristics and the course of this disease. The charts of 35 patients with either laryngeal (14 patients), tracheobronchial (10 patients), colonic (1 patient), or lower urinary tract amyloidosis (10 patients) were analyzed. The average age at diagnosis was 52.7 ± 12 years (range 33-73 years). The amyloid protein Type was specified to be amyloid light chain (AL) in 15 cases. All patients had undergone additional biopsies (accessory salivary glands, rectal, fat pad and bone marrow aspirates) to rule out a systemic disease. Symptomatic treatments included endoscopic excision and laser therapy. Colchicine and chemotherapy with prednisone and melphalan were prescribed with limited success. During a mean follow-up period of 6.1 ± 5.3 years no patient developed a systemic form of amyloidosis. Six deaths were reported, one related to the disease because of a fatal airway hemorrhage.
We suggest that immunolabeling studies should be more routinely performed. There was no risk of developing a systemic disease from local amyloid deposits in our survey. However, local evolution can be life-threatening. Such patients should be referred to specialist centers for further evaluation. Management requires close follow-up to exclude recurrence and to determine the appropriate symptomatic treatment.
Introduction
Amyloidosis is characterized by extracellular fibrillar protein deposits that display Congo-red staining and pathognomonic birefringence under cross-polarized light related to the β-sheet content. Localized amyloidosis has been described in almost every organ system [1-7]. Some patients have a progressive form of the disease that is associated with difficult management and severe morbidity. Evolution to a systemic disease remains unclear. No series has yet been reported in France. We analyzed the clinical characteristics, management and outcome of patients with localized amyloidosis, through a nationwide inquiry.
Patients and methods
From January 2001 to December 2003, we proposed a study supported by the Société Française de Médecine Interne (SNFMI). Every six months, through the annual meetings of the SNFMI, colleagues from departments of academic and general hospitals were motivated to include patients with localized amyloidosis. The patients had to present with localized amyloidosis affecting either the larynx, or the sinonasal cavities, or the upper respiratory or genito-urinary tracts,...