Abstract

Aim: To determine the clinicopathological properties of ocular adnexal marginal zone B cell lymphomas (MZBLs) with CD5+ B cells.

Methods: This study determined the clinicopathological properties of MZBL samples from 97 patients with ocular adnexal MZBLs and searched for hallmarks of systemic autoimmunity in these patients.

Results: Two elderly female patients were found to have ocular adnexal MZBLs with CD5+ B cells; flow cytometry analysis suggested that one of these MZBLs had CD5+ B cell clonal proliferation. The levels of anti-single stranded (SS)-DNA and anti-SS-A/Ro antibodies in these two patients were significantly higher than those in controls that were matched for age, gender and disease (2/2 versus 0/14; p = 0.008) and controls without MZBL (2/2 versus 0/30; p = 0.002). The genes from the immunoglobulin heavy-chain variable region for one of the patients showed a V3-21 segment. In addition, another patient with ocular adnexal reactive lymphoid hyperplasia with CD5+ B cells also had anti-SS-DNA antibodies.

Conclusion: Patients with ocular adnexal MZBLs with CD5+ B cells may have a background of systemic conditions with CD5+ B-cell-related autoantibodies.