Orbital Lymphoma in the Setting of Idiopathic CD4+Lymphocytopenia (HIV-Negative AIDS)

A 43-year-old Asian man with idiopathic CD4+ lymphocytopenia (human immunodeficiency virus [HIV]-negative acquired immunodeficiency syndrome [AIDS]) noted blurred vision OD. Visual acuity was 20/30 OD and 20/15 OS. The right eye showed features of axial proptosis (2 mm), blepharoptosis, upgaze restriction, conjunctival chemosis, color desaturation, and choroidal folds. The OS was unremarkable. There was no lymphadenopathy. Orbital MRI showed a well-circumscribed mass indenting the globe. Excision revealed diffuse large-B-cell lymphoma. Systemic evaluation was negative and adjuvant external beam radiotherapy to the right orbit was performed. Idiopathic CD4+ lymphocytopenia is a rare hematologic condition characterized by depletion of CD4+ T cells in HIV-negative patients. This condition closely resembles AIDS but HIV serology is negative. Similar to AIDS, patients are at risk for opportunistic infections and neoplasia, including systemic lymphoma. This is the first case, to the authors' knowledge, of orbital lymphoma with idiopathic CD4+ lymphocytopenia.