Lymphomatoid granulomatosis (LYG) is a rare angio-destructive
lymphoproliferative disorder (LPD) of uncertain etiology, with prominent pulmonary involvement. Recent studies indicate that LYG is an Epstein-Barr virus (EBV)-associated B cell LPD with large numbers of background reactive T lymphocytes (T cell-rich
B cell lymphoma). Although the disease frequently, but not exclusively, occurs in various immunodeficiency states, it has not been reported in association with the transient immunosuppression following autologous bone marrow/
peripheral stem cell transplantation (ABM/PSCT). We describe a patient who developed
lymphomatoid granulomatosis of the lung approximately 2 weeks after high-dose
chemotherapy and autologous
peripheral stem cell transplantation for
multiple myeloma. Although molecular studies showed no evidence of EBV genome in the biopsy material, the serologic profile with high
IgM titers was suggestive of primary
EBV infection. Complete radiologic remission occurred following reconstitution of the patient's immune response after a 2-week course of
ganciclovir treatment. Despite the apparently low frequency of LPD (both LYG and EBV-associated post-transplant
lymphoma) in the ABMT setting, we believe that it should be considered in the differential diagnosis of patients whose
clinical course following ABMT is complicated by
fevers, in the absence of an identifiable infectious process.