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Case Reports
. 2005 Feb 1;105(3):994-6.
doi: 10.1182/blood-2004-07-2965. Epub 2004 Oct 19.

Treatment of Primary Epstein-Barr Virus Infection in Patients With X-linked Lymphoproliferative Disease Using B-cell-directed Therapy

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Case Reports

Treatment of Primary Epstein-Barr Virus Infection in Patients With X-linked Lymphoproliferative Disease Using B-cell-directed Therapy

Michael C Milone et al. Blood. .

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.

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