Background: Lymphomatoid granulomatosis (LG) is an Epstein-Barr virus–associated, multisystemic disease that combines a granulomatous inflammatory process with lymphoproliferative potential. It is a rare disorder with a variable clinical presentation ranging from an indolent process to an aggressive B-cell lymphoma. Outcome is unpredictable, and a standard treatment has not yet been established. Cases treated with rituximab, an anti-CD20 monoclonal antibody, have been reported with variable results.
Observation: We report on 2 children with LG treated with rituximab and review the literature. The first patient had good response but the second did not.
Conclusions: Rituximab is a treatment option for LG.