Lymphomatoid granulomatosis following autologous stem cell transplantation

Lymphomatoid granulomatosis (LYG) is a rare angiodestructive lymphoprolifer ative disorder (LPD) of uncertain etiology, with prominent pulmonary involv ement, Recent studies indicate that LYG is an Epstein-Barr virus (EBV)-asso ciated B cell LPD with large numbers of background reactive T lymphocytes ( T cell-rich B cell lymphoma). Although the disease frequently, but not excl usively, occurs in various immunodeficiency states, it has not been reporte d 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 t ransplantation for multiple myeloma, Although molecular studies showed no e vidence of EBV genome in the biopsy material, the serologic profile with hi gh IgM titers was suggestive of primary EBV infection. Complete radiologic remission occurred following reconstitution of the patient's immune respons e after a 2-week course of ganciclovir treatment. Despite the apparently lo w frequency of LPD (both LYG and EBV-associated post-transplant lymphoma) i n the ABMT setting, we believe that it should be considered in the differen tial diagnosis of patients whose clinical course following ABMT is complica ted by fevers, in the absence of an identifiable infectious process.