Table of Contents

HK J Paediatr (New Series)
Vol 11. No. 3, 2006

HK J Paediatr (New Series) 2006;11:210-214

Immunology & Infectious Diseases Forum

Successful Treatment of X-Linked Lymphoproliferative Disease (XLP) with Anti-CD20 Monoclonal Antibody (Rituximab) Followed by Mismatched Unrelated Cord Blood Transplantation

TL Lee, HKW Law, GCF Chan, SY Ha, MHK Ho, KW Chan, YL Lau


A 4-year-old boy with X-linked lymphoproliferative disease (XLP) developed life-threatening acute lymphoproliferative crisis and failed to respond to conventional treatment of dexamethasone and etoposide. With the knowledge that uncontrolled alloreactive cytotoxic T-cell responses triggered by EBV-transformed B cells is the main cause of XLP, anti-CD20 monoclonocal antibody (Rituximab) which directed against B lymphocytes was use to damp down the patient's dysregulated immune response. He responded well to this novel approach and entered into complete remission with this treatment. His inherited immuno-deficient genetic defect was subsequently corrected by unrelated cord blood transplantation.

Keyword : Anti-CD20 monoclonal antibody; Cord blood transplantation; Rituximab; X-linked lymphoproliferative disease

Abstract in Chinese


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