Ocular manifestations of adult T-cell leukemia/lymphoma. A clinicopathologic study

Adult T-cell leukemia/lymphoma (ATLL) is a recently described distinct clinicopathologic entity characterized by an extremely aggressive clinical course, a leukemic or lymphomatous proliferation of hyperlobulated peripheral T cells, and an association with infection by a retrovirus, human T-lymphotropic virus type I (HTLV-I). The authors present a patient with ATLL who initially had clinical features of severe uniocular intraocular inflammation, simulating acute retinal necrosis or herpetic retinitis, but results of histopathologic examination of the globe showed ATLL.

A 38-year-old man had severe unilateral intraocular inflammation associated with widespread diffuse retinitis. Two weeks later, a focal retinitis with vasculitis developed nasal to the optic disc in the opposite eye. Shortly thereafter, mental disturbance developed, and the patient died less than 3 months after onset of the ocular symptoms.

Results of postmortem examination showed atypical lymphocytic infiltration in the eyes, brain, and other systemic organs. Results of histopathologic and immunohistochemical studies showed that the atypical cells infiltrating the ocular tissues were ATLL cells.

Patients infected with HTLV-I initially can have intraocular T-cell lymphoma that simulates acute retinal necrosis or herpetic retinitis.