A case of pulmonary lymphomatoid granulomatosis successfully treated by combination chemotherapy with rituximab.

Lymphomatoid granulomatosis (LYG) is a rare angiocentric and angiodestructive lymphoproliferative disease characterized by Epstein-Barr virus-positive B cells admixed with reactive T cells. LYG most commonly affects the lung but can also involve other extrapulmonary sites. Pulmonary LYG usually presents as multiple pulmonary nodules with rapid progression and excavation. It can mimic various infectious diseases, vasculitis or metastatic malignancy and is difficult to be diagnosed clinically. Standard treatment for LYG has not yet been established. Despite combination chemotherapy, the overall prognosis is poor. Recently, anti-CD20 monoclonal antibody, rituximab, has been used to treat LYG. We report the case of a 70-year-old male patient with pulmonary LYG, who showed rapid remission of the disease after combination chemotherapy with rituximab. Copyright 2009 S. Karger AG, Basel.