Rituxan for XLP – pro

Anti-CD20 (Rituximab) is a humanised monoclonocal antibody directed against the specific CD20 antigens found on B lymphocytes plasma membrane. It has been approved by the FDA for the treatment of lymphoma and post-transplant lymphoproliferative diseases. Its uses in several autoimmune diseases have recently been explored. The rationale for using Rituxan for treating EBV infections in children is that it can destroy B cells and thus “dampen” T-cell response caused by immune dysregulation. After primary infection, patients commonly develop a hemophagocytic syndrome known as fulminant infectious mononucleosis (FIM), which is characterized by the polyclonal expansion of EBV-infected B cells and the dysregulated expansion and activation of T cells and macrophages. FIM leads to hepatic and bone marrow (BM) failure and is fatal in more than 92% of cases.

This is a rare condition and rendomized studies are not possible. The case reports that have been published sugest that it is an effective treatment in an otherwise almost invariably fatal condition.

B.J. Mark, A.P. Knutsen Rituximab Treatment to Control Epstein-Barr Virus (EBV) Infection in X-Linked Lymphoproliferative Disorder (XLP)Volume 117, Issue 2, Supplement, Page S106 (February 2006)

TL Lee, HKW Law, GCF Chan, SY Ha, MHK Ho, KW Chan, YL Lau
Successful Treatment of X-Linked Lymphoproliferative Disease (XLP) with Anti-CD20 Monoclonal Antibody (Rituximab) Followed by Mismatched Unrelated Cord Blood Transplantation HK J Paediatr (New Series) 2006;11:210-214

Michael C. Milone, Donald E. Tsai, Richard L. Hodinka, Lewis B. Silverman, Alejandro
Blood, 1 February 2005, Vol. 105, No. 3, pp. 994-996.

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