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Hepatitis B virus associated localized laryngeal amyloidosis
*For correspondence: aanchalkakkar@gmail.com
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Received: ,
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This article was originally published by Wolters Kluwer - Medknow and was migrated to Scientific Scholar after the change of Publisher.
A 24 yr old male† non-smoker, farmer by occupation, presented to the Otorhinolaryngology outpatient clinic, All India Institute of Medical Sciences, New Delhi, India, in January 2018, with one-year history of progressive hoarseness. He had been diagnosed with chronic hepatitis B virus (HBV) infection (HBsAg +ve; HBeAg –ve; 2500 viral copies/ml plasma) three years earlier and was treated with tenofovir disoproxil 300 mg/day for six months. Treatment was stopped when <50 viral copies/ml plasma was achieved. Rest of the systemic examination was unremarkable. Fibre-optic laryngoscopic examination revealed a reddish vascular sub-mucosal bulge over the right false vocal cord, partially obliterating the airway lumen (Fig. 1A and Video). Contrast enhanced magnetic resonance imaging (CEMRI) of the neck revealed a post-contrast T1 hyperintense and T2 hypointense lesion involving the right supraglottic larynx (Fig. 1B and C). With a clinical diagnosis of benign laryngeal mass, he underwent complete excision of the same. Biopsy (Fig. 2) revealed that submucosal deposits of the extracellular amorphous eosinophilic material stained positively with Congo red, demonstrated apple green birefringence under polarized light, were immunopositive for serum amyloid-associated protein and lacked light chain restriction, indicating that the deposits were AA amyloid. After extensive work-up, a diagnosis of secondary localized laryngeal amyloidosis was made, with HBV infection as the possible underlying chronic inflammatory aetiology. He remained asymptomatic at 24 months' of follow up. The larynx is the most common site of amyloid deposition (usually is light chain type) in the head and neck. These lesions can have varied appearance, ranging from nodules to non-ulcerated, yellow, red or white lesions or a diffuse tissue mass. The treatment of choice is endoscopic excision and prognosis is good.
- (A) Endoscopic view of the larynx showing reddish bulge in the region of right false vocal cord (arrow). (B) Axial T2 and (C) post-contrast T1 images of the neck showing homogeneously enhancing T2 hypointense lesion involving left aryepiglottic fold and false vocal cord as evidenced by enhancing nodularity along the mucosa (arrows).
- (A) Laryngeal biopsy showing submucosal deposits of extracellular amorphous eosinophilic material (H and E, ×40, arrows), that (B) stain positively with Congo red (×100, arrow), that demonstrate (C) apple green birefringence (×200) on polarizing microscopy (arrow) and that are (D) immunopositive for serum amyloid-associated protein (×100, arrows). Immunofluorescence on paraffin sections for Kappa (E) and lambda (F) (×100) does not reveal light chain restriction.
Video available at ijmr.org.in.
Acknowledgment:
Authors acknowledge Dr Geetika Singh, department of Pathology, AIIMS, New Delhi for providing pathological images.
Conflicts of Interest: None.
