Diagnosis of a multicentric extranodal nasal-type natural killer T-cell lymphoma made with an anterior chamber tap.

PURPOSE: To report the case of an unusual multicentric Epstein-Barr virus (EBV)-associated extranodal nasal-type natural killer T-cell lymphoma masquerading as unilateral panuveitis, diagnosed with an anterior chamber tap.

METHODS

The clinical history and the morphological, immunohistochemical, and molecular features of a 51-year-old white man with left severe panuveitis were retrospectively evaluated.

RESULTS

The patient initially presented with a 3-month history of recalcitrant sinusitis and new onset of unilateral loss of vision. Clinical examination revealed right peripheral facial nerve palsy, severe panuveitis of the left eye, and nasopharyngeal obstruction. Anterior chamber aspirates were examined. The combination of the presence of small- to intermediate-size lymphocyte proliferation, moderate elevation of the interleukin-10 level on cytokine profiling, and slightly positive polymerase chain reaction for EBV in the aqueous humor indicated an EBV-induced nasal-type natural killer T-cell lymphoma. Transnasal biopsy revealed the presence of numerous irregular lymphoma cells with positive staining for CD3, CD56, EBV-encoded RNA in situ hybridization, and negative staining for CD4, CD8, and CD1a. Lumbar puncture, cerebral magnetic resonance imaging, thoracoabdominal computed tomography, and upper digestive tract endoscopy revealed meningeal, renal, adrenal, and digestive involvement. Massive hemorrhage of the upper digestive tract caused rapid death.

CONCLUSION

Extranodal nasal-type natural killer T-cell lymphoma is a very uncommon disease that may present acutely, sometimes as pseudouveitis. Simple investigations such as anterior chamber aspirates for cytological examination, reinforced by cytokine profiling and viral polymerase chain reaction looking for EBV, may provide a rapid diagnosis, necessary given the poor prognosis of the disease. To our knowledge, and after extensive review of the literature, we did not find another case report diagnosing this entity by anterior chamber paracentesis.