title: Treatment of primary Epstein-Barr virus infection in patients with X-linked lymphoproliferative disease using B-cell-directed therapy.
abstract: X-linked lymphoproliferative disease (XLP) is a congenital immunodeficiency that is characterized by an abnormal immune response to primary Epstein-Barr virus (EBV) infection. After EBV exposure, affected patients often develop fulminant infectious mononucleosis (FIM), a life-threatening condition marked by the uncontrolled expansion and activation of T and B lymphocytes and macrophages. We hypothesized that the rapid elimination of B cells immediately following EBV exposure might reduce the severity of primary EBV infection in patients with XLP. To test this possibility, we administered the anti-CD20 antibody rituximab to 2 patients who presented with acute infection. Following treatment, both patients exhibited a complete resolution of symptoms and no longer demonstrated detectable EBV DNA within circulating lymphocytes. Moreover, neither patient has developed FIM or lymphoma in more than 2 years of follow-up. These data suggest that the pre-emptive use of B-cell-directed therapy may reduce the morbidity and mortality of primary EBV infection in XLP-affected individuals.
authors.affiliation: Department of Pathology and Laboratory Medicine, Hematologic Malignancies Program, University of Pennsylvania, Philadelphia, USA.
authors.forename: Michael C
authors.initials: MC
authors.lastname: Milone
authors.forename: Donald E
authors.initials: DE
authors.lastname: Tsai
authors.forename: Richard L
authors.initials: RL
authors.lastname: Hodinka
authors.forename: Lewis B
authors.initials: LB
authors.lastname: Silverman
authors.forename: Alejandro
authors.initials: A
authors.lastname: Malbran
authors.forename: Mariusz A
authors.initials: MA
authors.lastname: Wasik
authors.forename: Kim E
authors.initials: KE
authors.lastname: Nichols
mesh_terms: D000328:Adult; D001402:B-Lymphocytes / Q000276:immunology; D041321:Chromosomes, Human, X; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000628:therapy*; D006801:Humans; D008232:Lymphoproliferative Disorders / Q000150:complications* / Q000235:genetics; D008297:Male; D033581:Stem Cell Transplantation