Primary CD4 + T-Cell Lymphoproliferative Disorder of Conjunctiva

The purpose of this study was to describe a patient with a primary CD4 + T-cell lymphoproliferative disorder of conjunctiva.

Case report and review of the literature.

An 81-year-old man was referred for asymptomatic bilateral conjunctival masses that on biopsy revealed a proliferation of mature CD4 + lymphocytes associated with T-cell receptor γ gene rearrangement. The histopathology and immunophenotypic profile in the absence of disease elsewhere bore resemblance to primary cutaneous CD4 + small/medium T-cell lymphoproliferative disorder.

In 2016, the World Health Organization reclassified primary cutaneous CD4+ small/medium T-cell lymphoma to a lymphoproliferative disorder because neither dissemination nor death had been documented. This case displays similarities with the latter but has nonconforming features. Multicentric clonal expansion of T cells do not inevitably indicate lymphoma. Lymphoproliferative disorders that display features of reactive hyperplasia and lymphoma and are not easily classified need to be carefully monitored.