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Extranodal Natural Killer/T-Cell Lymphoma (ENKTL) Malignant Pleural Effusion: A Rare Finding in an Uncommon Disease FREE TO VIEW

Richard May, MD; Henry Dinneen, DO; Andrew Berman, MD
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New Jersey Medical School - Rutgers, Newark, NJ

Chest. 2014;146(4_MeetingAbstracts):648A. doi:10.1378/chest.1980642
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SESSION TITLE: Cancer Student/Resident Case Report Posters I

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Extranodal natural killer/T-cell lymphoma (ENKTL), nasal type, is a rare form of non-hodgkin lymphoma commonly affecting the paranasal region. Extranasal pulmonary involvement is extremely rare. We report a case of ENKTL initially presenting with bilateral pleural effusions.

CASE PRESENTATION: A 34 year old female from the Caribbean with no past medical history presented with progressive shortness of breath for three months, associated with a cough productive of yellow sputum. She reported subjective fevers and a five pound unintentional weight loss. On examination, she was febrile at 102.3F and tachypneic, with a room air oxygen saturation of 96%. Diminished breath sounds were noted at both posterior lung bases. A firm, non-tender 5x3cm ulcerated right axillary mass was found. Laboratory studies revealed leukocytosis with a left shift and lactate dehydrogenase was 1623 units/L. Chest radiography showed a large right pleural effusion. Computed tomography of the chest showed bilateral pleural effusions, pleural nodularity and multifocal patchy opacities. She was started on empiric antibiotic coverage with vancomycin and piperacillin-tazobactam. Thoracentesis revealed straw colored exudative fluid. Cytology of the pleural fluid showed granular appearing lymphocytes. Immunostaining was positive for CD45, CD3, and CD56, with reactivity to Ki-67. Quantiferon Gold test was negative. Right axillary mass excisional biopsy had atypical lymphoid cells. Immunostaining was positive for CD2, CD56, CD3, C7, CD8, CD30, Granzyme B, TIA-1, and perforin. Ki-67 was present at 90%, and EBV in situ hybridization was strongly positive. These findings were consistent with ENKTL. Hospital course was complicated by a pneumothorax after the thoracentesis requiring chest tube placement. The patient then elected for comfort care only and expired 10 days after admission.

DISCUSSION: Primary pulmonary involvement of ENKTL is an exceedingly rare presentation of this aggressive form of lymphoma. Most patients present with localized disease. The upper airway is sometimes involved due to direct extension of the primary tumor. Review of the published literature reveals one case report of ENKTL initially presenting with pleural effusions. Patients with extranasal disease have a poor prognosis despite systemic chemotherapy with survival measured in months. Ki-67 over 50%, as in our patient, also predicts poor prognosis. Early involvement by Palliative Care services is warranted in patients presenting with advanced ENKTL.

CONCLUSIONS: ENKTL is a rare aggressive form of non-hodgkin lymphoma. Pleuropulmonary involvement is a rare manifestation of this disease. Prognosis is extremely poor.

Reference #1: Clinical differences between nasal and extranasal natural killer/T-cell lymphoma: a study of 136 cases from the International Peripheral T-Cell Lymphoma Project. Au, W et al. Blood 2009; 113: 3931-3937.

DISCLOSURE: The following authors have nothing to disclose: Richard May, Henry Dinneen, Andrew Berman

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