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A6450 - Not Your Ordinary Sore Throat: A Rare Case of Extranodal Lymphoma
Author Block: J. Lyou1, T. T. Cheng2, N. Wahid3, J. Longoria4, M. Davoudi5; 1Pulmonary, University California, Irvine, Orange, CA, United States, 2Pulmonary, University of California, Irvine, Orange, CA, United States, 3Internal Medicine, University California, Irvine, Orange, CA, United States, 4UCSD, San Diego, CA, United States, 5Pulm Crit Care Div, UCSD, San Diego, CA, United States.
Introduction
Extranodal natural killer/T cell lymphoma, nasal type (ENKL) is a rare but aggressive type of non-Hodgkin’s lymphoma that is strongly associated with Ebstein-Barr virus (EBV) infection, with higher incidence in Hispanic Whites and Asian/Pacific Islanders. Clinical presentation is highly variable and may present with airway compromise, epistaxis, or nasal obstruction. We describe a rare case of nasal-type NK/T cell lymphoma in a young previously healthy young female.
Case Presentation
A 23-year-old Hispanic female presented to the emergency room with dyspnea, productive cough, and hoarseness for three months. She also described three days of nausea and fever up to 104 degrees Fahrenheit. In the months prior to admission, the patient presented to several urgent care clinics and was treated empirically for acute bronchitis with multiple courses of oral steroids which provided temporary relief of her respiratory symptoms. Computed tomography (CT) neck showed bilateral symmetric thickening of the vocal folds and thyroarytenoid muscles. Flexible bronchoscopy performed under moderate sedation revealed edematous nasal turbinates and multiple 1-2 millimeter friable nodules diffusely along the true and false vocal cords, terminating immediately above the subglottic funnel. Serologies for vasculitis, rheumatologic disorders, and infectious etiologies (typical and atypical bacterial, viral, and fungal) were negative. HIV testing was negative. Patient was brought to the operating room to proceed with rigid bronchoscopy under general anesthesia for both diagnostic and therapeutic purposes. The glottis and subglottis were dilated to about 10 mm and a biopsy sample was taken from the abnormal tissue. Subsequent surveillance flexible bronchoscopies performed during the following weeks showed significant improvement of the edematous mucosa at the vocal cords to the level of the cricoid cartilage. The subglottic tissue biopsy results unexpectedly revealed diffuse proliferation of atypical lymphoid cells with overlying ulceration and necrosis. Biopsy was positive for CD2, CD3, CD7, CD30, CD56, and ISH-EBV with high proliferation index by Ki-67. These results were consistent with nasal-type extranodal NK/T cell lymphoma.
Discussion
Diagnosis of ENKL requires biopsy of tissue specimen from the affected site. Our patient’s presentation proved to be a diagnostic and therapeutic challenge given the location of the tumors and progressive edema at the vocal cords, which placed the patient at risk for airway compromise. Interventional pulmonary procedural techniques were utilized to obtain tissue and dilate the glottis without the need for an emergent tracheostomy in this young female patient.