A 22‐year‐old El Salvadoran man presented with 2 months of fevers, night sweats, fatigue, cough with scant hemoptysis, hoarseness and a 60‐pound weight loss. Over the next month, he developed progressive dysphagia, odynophagia, and dyspnea. On presentation, he had a temperature of 103.3°F oxygen saturation of 94% on room air and respiratory rate of 24. His exam was notable for crepitus around the neck. Laboratory findings were significant for hemoglobin 11.5 g/dL, ESR 81 mm/h, and CRP 164 mg/L. X‐rays and CT scans of the neck and chest demonstrated a pneumomediastinum, retropharyngeal and subcutaneous emphysema, and lung parenchyma with diffuse interstitial edema. On direct laryngoscopy, the epiglottis was erythematous and asymmetric with extensive polypoid changes. There was also extensive subepithelial emphysema with mucosal fungation and cobblestoning in the retropharynx. The patient was started on empiric treatment for laryngeal tuberculosis. Multiple sputum samples were negative for acid‐fast bacilli. Epstein–Barr virus (EBV) capsid IgG, nuclear antibody, and early antibody titers were markedly elevated. On hospital day 6, an epiglottal biopsy was performed. Pathological findings were consistent with an extranodal natural killer/T‐cell lymphoma, nasal type with T‐cell receptor gene rearrangements, and expression of EBV markers. Antituberculosis drugs were discontinued. PET‐CT scan demonstrated hypermetabolic signals in the nasopharynx, oropharynx, and cervical lymph nodes consistent with malignancy. The patient is currently undergoing combined radiation and chemotherapy with dexamethasone, etoposide, ifosfamide, and carboplatin.
Peripheral T‐cell lymphomas account for less than 10%–15% of all non‐Hodgkin's lymphoma, of which only 10% are extranodal NK/T‐cell lymphoma, nasal type. This is an uncommon and highly aggressive lymphoma, more common in Asia and native populations of Central and South America and is closely associated with EBV infection. It often presents with prominent B‐symptoms, nasal obstruction, and epistaxis. The most severe cases are complicated by invasion of the mid‐acial structures and upper aerodigestive tract that may lead to respiratory compromise. This condition is highly responsive to radiation therapy if confined to local disease, with relapse rates improved when treated in combination with chemotherapy.
The purpose of this case is to increase awareness of this rare disease and its potentially acute pulmonary complications.