Abnormal expression of CD20 on IgG4 plasma cells associated with IgG4-related lymphadenopathy.

CONTEXT: Immunoglobulin G4 (IgG4)-related disease is a recently described entity that presents as mass-forming lesions in soft tissue, exocrine glands, and in lymph nodes as IgG4-related lymphadenopathy. The underlying pathologic mechanism of IgG4-related disease is unclear; however, rituximab (an anti-CD20 monoclonal antibody) has been shown to have clinical efficacy.

OBJECTIVE

To look for the presence or absence of CD20 on the IgG4-expressing plasma cells in IgG4-related lymphadenopathy.

DESIGN

Twelve flow cytometry cases were identified through a retrospective review from the authors' institutions files. Cases were selected by the presence of a lymph node biopsy specimen with increased IgG4 plasma cells by immunohistochemistry and a histologic diagnosis compatible with IgG4-related lymphadenopathy.

RESULTS

We report dim CD20 expression on plasma cells in all cases for which a plasma cell population was clearly identified by flow cytometry. These cases were from patients with lymph node biopsy specimens that met published criteria for IgG4-related lymphadenopathy.

CONCLUSIONS

This finding may be one potential explanation for the clinical efficacy of rituximab in IgG4-related disease.