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Diagnostic dilemma in a rare case of extranodal natural killer T-Cell lymphoma

Lee, J., Moynihan, V., Ardakani, N. and Gajdatsy, A. (2022) Diagnostic dilemma in a rare case of extranodal natural killer T-Cell lymphoma. Clinical & Experimental Opthalmology, 50 (8). pp. 938-939.

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Purpose: To describe a rare case of natural killer T-Cell lymphoma complicated by secondary haemophagocytic lymphohistiocytosis (HLH) presenting with unilateral orbital inflammation and undifferentiated systemic inflammatory disease.

Method: A review of medical records, imaging and histopathology.

Result: A 61-year-old male was referred with a ten-day history of right periorbital swelling. He had a history of lower respiratory tract infection prior with ongoing symptoms of breathlessness and dry cough. Imaging demonstrated fusiform enlargement of the medial and inferior rectus muscles and moderate proptosis. Mildly elevated inflammatory markers and deranged liver function tests were also noted on admission. After being initially treated for presumed orbital inflammatory syndrome, he subsequently developed an orbital compartment syndrome requiring a canthotomy and cantholysis and two endonasal decompressions. He developed acute respiratory distress syndrome on day 7 requiring intubation and management by intensive care. The presence of lympohistiocytic infiltrates in an orbital fat biopsy, fever, progressive pulmonary infiltrates, worsening liver function tests, pancytopenia and elevated ferritin confirmed the diagnosis of HLH (Modified 2009 HLH Criteria). A definitive pathologic diagnosis was made of an extranodal NK/T-Cell lymphoma (Nasal Type, EBV positive). The patient died on day 26 of admission secondary to multiple complications of HLH and chemotherapy-induced ileitis.

Conclusion: Natural Killer T-Cell lymphoma is a rare but aggressive and devastating lymphoma comprising only 1–3% of all orbital Non-Hodgkin Lymphomas. HLH is an uncommon multi-system inflammatory complication which can be triggered by lymphoid malignancies. This is the first reported case of an orbital NK/T-Cell Lymphoma presenting with HLH.

Item Type: Journal Article
Publisher: Wiley
Copyright: © 2022 Royal Australian and New Zealand College of Ophthalmologists.
Other Information: Poster abstract given @ The Royal Australian and New Zealand College of Ophthalmologists 53rd Annual Scientific Congress 28 October – 1 November 2022
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