What is the role of hematopoietic stem cell transplantation (HSCT) in the treatment of Chédiak-Higashi syndrome (CHS)?

Updated: Aug 08, 2019
  • Author: Roman J Nowicki, MD, PhD; Chief Editor: Dirk M Elston, MD  more...
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Allogeneic transplantation from an HLA-matched sibling or from an unrelated donor or cord blood transplantation is the treatment of choice to correct the immunologic and hematologic manifestations of early-onset Chédiak-Higashi syndrome (CHS). Transplantation appears to be most successful when performed prior to the accelerated phase. Therefore, it would be of great value to differentiate patients who present with the childhood form of the disease from those who exhibit clinical phenotypes of adolescent and adult CHS, so as to prematurely enroll only the former ones in to a transplantation protocol. [14]

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