Case report Lymphomatoid granulomatosis following autologous stem cell transplantation

Summary:Lymphomatoid granulomatosis (LYG) is a rare angio-destructive lymphoproliferative disorder (LPD) ofuncertain etiology, with prominent pulmonary involve-ment. Recent studies indicate that LYG is an Epstein–Barr virus (EBV)-associated B cell LPD with large num-bers of background reactive T lymphocytes (T cell-richB cell lymphoma). Although the disease frequently, butnot exclusively, occurs in various immunodeficiencystates, it has not been reported in association with thetransient immunosuppression following autologousbone marrow/peripheral stem cell transplantation(ABM/PSCT). We describe a patient who developedlymphomatoid granulomatosis of the lung approxi-mately 2 weeks after high-dose chemotherapy and auto-logous peripheral stem cell transplantation for multiplemyeloma. Although molecular studies showed noevidence of EBV genome in the biopsy material, theserologic profile with high IgM titers was suggestive ofprimary EBV infection. Complete radiologic remissionoccurred following reconstitution of the patient’simmune response after a 2-week course of ganciclovirtreatment. Despite the apparently low frequency of LPD(both LYG and EBV-associated post-transplantlymphoma) in the ABMT setting, we believe that itshould be considered in the differential diagnosis ofpatients whose clinical course following ABMT is com-plicated by fevers, in the absence of an identifiable infec-tious process.Keywords: EBV; lymphomatoid granulomatosis; auto-logous transplantation; immunosuppressionLymphomatoid granulomatosis, initially described by Lie-bow in 1972, is characterized histologically by a polymor-phic lymphoid infiltrate with varying degrees of necrosis