گزارش يك مورد آميلوييدوز سيستميك در حنجره

A Case of Systemic Amyloidosis with Primary Manifestation in Larynx: Diagnosis, cPathophysiorogy and Treatment

Introduction: Amyloidosis is an idiopathic disorder characterized by amyloid deposition leading to tissue damage and disease. Laryngeal amyloidosis is usually a localized phenomenon that is rarely accompanied by systemic involvement. Hoarseness is its most common symptom and the clinical findings in laryngoscopy are variable, nonspecific and difficult to be distinguished from other laryngeal lesions. The diagnosis is made by pathological examination and the treatment consists traditionally of surgical excision to maintain a functional airway and optimize voice. Considering the rarity of systemic involvement with laryngeal amyloidosis, this article presents a case of systemic amyloidosis with primary manifestation in the larynx. Case Report: The case was a 45-year-old man referred to ENT ward of Firouzgar Hospital with primary chief complaint of dyspnea and hoarseness. Primary biopsy from endolarynx indicated an inflammatory process and two later biopsies revealed inflammation and submucosal hemangioma as well. Finally, in the last laryngoscopy and biopsy with Congo-red staining, amyloid deposits were observed and laryngeal amyloidosis was documented. Additional biopsies from gingival mucous and abdominal fat and the presence of amyloid deposits in these sites confirmed the diagnosis of systemic amyloidosis. Conclusion: Systemic amyloidosis with laryngeal involvement is a rare disorder which is currently treated by serial direct laryngoscopy and microsurgery. Research on its medical treatment is ongoing.