Epstein-Barr virus polymorphic B-cell lymphoma associated with leukemia and with congenital immunodeficiencies.

The association of Burkitt’s lymphoma and nasopharyngeal carcinoma with the Epstein-Barr virus (EBV) is well known. In recent years other EBV associated tumors, such as diffuse polymorphic lymphomas have been seen in renal transplant patients by Hanto and others (1) and in various organ transplant patients by Starzl and others (2). Similar tumors have occasionally been reported in patients with congenital and acquired immunodeficiencies particularly in patients with the acquired immunodeficiency syndrome (AIDS) caused by the human immunodeficiency virus (3–6). Such tumors have been reported in a patient with leukemia (7) and in an apparently normal individual (8). We have observed this tumor in 2 children on maintenance therapy for leukemia and in 4 children with congenital immunodeficiencies. The Epstein-Barr virus (EBV) nuclear antigen (EBNA) was detected in the tumor cells and/or DNA from these was positive for EBV by molecular hybridization. An EBV positive culture could be obtained from biopsied tumors but not from tumor tissue removed at autopsy.