Atypical Hyperplasia of the Marginal Zone of B Follicles in a Polymorphic Epstein-Barr Virus–Associated Lymphoproliferative Disorder Occurring in an Adolescent with Human Immunodeficiency Virus Infection

Abstract

Epstein-Barr virus (EBV)-associated lymphoid proliferations that are similar to post-transplantation lymphoproliferative disorders may occasionally occur in the setting of human immunodeficiency virus (HIV) infection. Herein, we describe such a lesion involving the adenoids in a HIV-seropositive adolescent who acquired immunity against EBV during childhood. On microscopic examination, the marginal zone of B follicles and the interfollicular area were enlarged due to the accumulation of small or intermediate-sized lymphocytes, immunoblasts, epithelioid histiocytes, and plasma cells. A few atypical immunoblasts resembling Reed-Sternberg cells were also present. Most of the cells seen in these expanded regions belonged to the B-cell lineage and displayed a phenotype consistent with that of postgerminal center B cells. No clonal rearrangement of the genes coding for the heavy chain of the immunoglobulin could be demonstrated by polymerase chain reaction analysis. In-situ hybridization studies revealed the presence of EBV early RNA in a significant number of these cells, which suggests the participation of this virus in the pathogenesis of such a B-cell proliferation. The clinical course was benign; no progression or recurrence could be seen more than 24 months after the diagnosis. This atypical lymphoproliferative disorder is probably related to polyclonal reactivation of a latent EBV infection due to a local or systemic immune imbalance induced by HIV replication. Recognition of this reactive condition is important to prevent overtreatment.