Vision-threatening complications of nasal T/NK lymphoma

Nasal T/NK lineage lymphoma, previously known as lethal midline granuloma, is common among Oriental, Native-American, and Hispanic patients and is invariably associated with Epstein-Barr virus. Nasal localization, local necrosis, angioinvasion, and aggressive behavior are hallmarks of the disease. Ophthalmologic symptoms and signs may precede lymphoma diagnosis or complicate its disease course. We aim to define the incidence and disease pattern of ophthalmic involvement.

Retrospective single-institution consecutive interventional case series.

Records of all lymphoma patients from 1996 to 2000 were retrieved from a computer database, and the primary sites and lineage were reviewed. Nasal T/NK lymphoma was defined by a combination of morphology, positive CD3 epsilon and CD56 expression, in situ hybridization staining for Epstein-Barr virus expressed RNA (EBER), and negative staining for B cell markers (CD20 and CD79a).

Thirty-five consecutive patients with T/NK lymphoma were identified. Primary nasal or nasopharyngeal disease was found in 24 patients. Six (25%) of the 24 patients suffered from vision-threatening complications of two distinct categories, namely uveitis/vitritis and orbital infiltration. The former preceded the diagnosis of lymphoma in two patients and also led to novel ocular complications like rhegmatogenous retinal detachment and macular hole that were successfully managed by ocular surgery. In four patients, orbital extension of lymphomatous disease accompanied biopsy-proven in-field disease relapse. Generally, the prognosis is grave, but remission can still be achieved with aggressive combined chemotherapy and radiotherapy.

Both oncologists and ophthalmologists should be aware of ocular complications when dealing with known or suspected cases of T/NK lymphoma at diagnosis and relapse. Regular ophthalmic assessment of these patients is warranted.