Enlarged tongue due to primary systemic amyloidosis: clinicopathologic observation

摘要

Primary amyloidosis (AL) is characterized by deposition of abnormal extra cellular protein in the form of fibrils in many organs, especially the heart, kidneys, gastrointestinal tract, and peripheral nervous system. Involvement of the tongue is not uncommon in primary AL In 22 % to 26 % of patients suffering from AL, amyloid deposition in the tongue can result in an enlarged tongue. Lingual changes arising from localization or from all over, AL has been reported frequently as initial signs of the disease. Macroscopically, it is difficult to distinguish AL of the tongue from other lesions. In view of the variety of protein species involved and the wide spectrum of possible clinical presentations, the diagnosis of lingual AL is frequently overlooked, because immunohistochemical studies of such cases have not been undertaken. Here we describe two male patients with primary systemic AL who developed enlarged tongues. In addition to the manifestation of lingual AL, in which oral signs were the primary indicators of the disease, we describe the immunohistochemical findings of the tongue to discuss diagnostic criteria for lingual amyloid in primary AL.