Viion-threatening complication of naal T/NK lymphoma

PURPOE: Naal T/NK lineage lymphoma, previouly known a lethal midline granuloma, i common among Oriental, Native-American, and Hipanic patient and i invariably aociated with Eptein-Barr viru. Naal localization, local necroi, angioinvaion, and aggreive behavior are hallmark of the dieae. Ophthalmologic ymptom and ign may precede lymphoma diagnoi or complicate it dieae coure. We aim to define the incidence and dieae pattern of ophthalmic involvement. DEIGN: Retropective ingle-intitution conecutive interventional cae erie. METHOD: Record of all lymphoma patient from 1996 to 2000 were retrieved from a computer databae, and the primary ite and lineage were reviewed. Naal T/NK lymphoma wa defined by a combination of morphology, poitive CD3 and CD56 expreion, in itu hybridization taining for Eptein-Barr viru expreed RNA (EBER), and negative taining for B cell marker (CD20 and CD79a). REULT: Thirty-five conecutive patient with T/NK lymphoma were identified. Primary naal or naopharyngeal dieae wa found in 24 patient. ix (25%) of the 24 patient uffered from viion-threatening complication of two ditinct categorie, namely uveiti/vitriti and orbital infiltration. The former preceded the diagnoi of lymphoma in two patient and alo led to novel ocular complication like rhegmatogenou retinal detachment and macular hole that were uccefully managed by ocular urgery. In four patient, orbital extenion of lymphomatou dieae accompanied biopy-proven in-field dieae relape. Generally, the prognoi i grave, but remiion can till be achieved with aggreive combined chemotherapy and radiotherapy. CONCLUION: Both oncologit and ophthalmologit hould be aware of ocular complication when dealing with known or upected cae of T/NK lymphoma at diagnoi and relape. Regular ophthalmic aement of thee patient i warranted.