Hematopoietic stem cell transplantation conditioning with use of rituximab in EBV related lymphoproliferative disorders

Clin Immunol. 2014 Apr;151(2):79-83. doi: 10.1016/j.clim.2014.01.007. Epub 2014 Feb 4.

Abstract

X-linked lymphoproliferative disease (XLP) and IL-2-inducible T cell kinase (ITK) deficiency are rare immunodeficiencies with a spectrum of clinical manifestations. Although there are no official guidelines for allogeneic hematopoietic stem cell transplantation (HSCT) in these patients, previous reports have shown that reduced intensity conditioning regimens provide successful engraftment with limited toxicity. Here, we report on three children with XLP and one with ITK deficiency, who underwent successful HSCT using a rituximab containing conditioning regimen, and review the current literature.

Keywords: Epstein Barr virus; ITK deficiency; Primary immunodeficiency; Rituximab; Stem cell transplantation; X-linked lymphoproliferative.

Publication types

  • Case Reports

MeSH terms

  • Antibodies, Monoclonal, Murine-Derived / pharmacology*
  • Antineoplastic Agents / therapeutic use
  • Bone Marrow Transplantation
  • Child
  • Child, Preschool
  • Graft vs Host Disease / prevention & control
  • Hematopoietic Stem Cell Transplantation / methods*
  • Herpesvirus 4, Human*
  • Humans
  • Immunologic Factors / pharmacology*
  • Immunosuppressive Agents / therapeutic use
  • Infant
  • Lymphoproliferative Disorders / genetics
  • Lymphoproliferative Disorders / therapy*
  • Lymphoproliferative Disorders / virology
  • Male
  • Protein-Tyrosine Kinases / deficiency
  • Rituximab
  • Transplantation Conditioning / methods*

Substances

  • Antibodies, Monoclonal, Murine-Derived
  • Antineoplastic Agents
  • Immunologic Factors
  • Immunosuppressive Agents
  • Rituximab
  • Protein-Tyrosine Kinases
  • emt protein-tyrosine kinase