Stem-cell gene therapy for the Wiskott-Aldrich syndrome

Research output: Contribution to journalResearch articleContributedpeer-review

Contributors

  • Kaan Boztug - , Hannover Medical School (MHH) (Author)
  • Manfred Schmidt - , German Cancer Research Center (DKFZ) (Author)
  • Adrian Schwarzer - , Hannover Medical School (MHH) (Author)
  • Pinaki P. Banerjee - , University of Pennsylvania (Author)
  • Inés Avedillo Díez - , Hannover Medical School (MHH) (Author)
  • Ricardo A. Dewey - , Hannover Medical School (MHH) (Author)
  • Marie Böhm - , Hannover Medical School (MHH) (Author)
  • Ali Nowrouzi - , German Cancer Research Center (DKFZ) (Author)
  • Claudia R. Ball - , German Cancer Research Center, partner site Dresden, Environmental Monitoring and Endocrinology (Research Group), National Center for Tumor Diseases Dresden (Author)
  • Hanno Glimm - , German Cancer Research Center, partner site Dresden, National Center for Tumor Diseases Dresden (Author)
  • Sonja Naundorf - , BioNTech Ag (Author)
  • Klaus Kühlcke - , BioNTech Ag (Author)
  • Rainer Blasczyk - , Hannover Medical School (MHH) (Author)
  • Irina Kondratenko - , Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology (Author)
  • László Maródi - , University of Debrecen (Author)
  • Jordan S. Orange - , University of Pennsylvania (Author)
  • Christof Von Kalle - , German Cancer Research Center (DKFZ) (Author)
  • Christoph Klein - , Hannover Medical School (MHH) (Author)

Abstract

The Wiskott-Aldrich syndrome (WAS) is an X-linked recessive primary immunodeficiency disorder associated with thrombocytopenia, eczema, and autoimmunity. We treated two patients who had this disorder with a transfusion of autologous, genetically modified hematopoietic stem cells (HSC). We found sustained expression of WAS protein expression in HSC, lymphoid and myeloid cells, and platelets after gene therapy. T and B cells, natural killer (NK) cells, and monocytes were functionally corrected. After treatment, the patients' clinical condition markedly improved, with resolution of hemorrhagic diathesis, eczema, autoimmunity, and predisposition to severe infection. Comprehensive insertion-site analysis showed vector integration that targeted multiple genes controlling growth and immunologic responses in a persistently polyclonal hematopoiesis. (Funded by Deutsche Forschungsgemeinschaft and others; German Clinical Trials Register number, DRKS00000330.).

Details

Original languageEnglish
Pages (from-to)1918-1927
Number of pages10
JournalNew England Journal of Medicine
Volume363
Issue number20
Publication statusPublished - 11 Nov 2010
Peer-reviewedYes

Keywords

ASJC Scopus subject areas