Gene therapy improves immune function in preadolescents with X-linked severe combined immunodeficiency

Blood. 2007 Jul 1;110(1):67-73. doi: 10.1182/blood-2006-11-058933. Epub 2007 Mar 16.

Abstract

Retroviral gene therapy can restore immunity to infants with X-linked severe combined immunodeficiency (XSCID) caused by mutations in the IL2RG gene encoding the common gamma chain (gammac) of receptors for interleukins 2 (IL-2), -4, -7, -9, -15, and -21. We investigated the safety and efficacy of gene therapy as salvage treatment for older XSCID children with inadequate immune reconstitution despite prior bone marrow transplant from a parent. Subjects received retrovirus-transduced autologous peripherally mobilized CD34(+) hematopoietic cells. T-cell function significantly improved in the youngest subject (age 10 years), and multilineage retroviral marking occurred in all 3 children.

Publication types

  • Clinical Trial
  • Research Support, N.I.H., Intramural

MeSH terms

  • Genetic Therapy / methods*
  • Hematopoietic Stem Cell Transplantation / methods
  • Humans
  • Immunity / drug effects*
  • Infant
  • Mutation
  • Receptors, Interleukin-2 / administration & dosage*
  • Receptors, Interleukin-2 / genetics
  • Retroviridae / genetics
  • T-Lymphocytes / immunology
  • Transduction, Genetic
  • Transplantation, Autologous
  • X-Linked Combined Immunodeficiency Diseases / genetics
  • X-Linked Combined Immunodeficiency Diseases / immunology*
  • X-Linked Combined Immunodeficiency Diseases / therapy*

Substances

  • Receptors, Interleukin-2