Hematopoietic stem cell transplantation for sickle cell disease: Progress and challenges

Pediatr Blood Cancer. 2018 Sep;65(9):e27263. doi: 10.1002/pbc.27263. Epub 2018 May 24.

Abstract

Sickle cell disease (SCD) presents challenges to hematopoietic stem cell transplantation (HSCT), including donor availability and morbidity with age/disease severity. However, severe SCD causes irreversible organ damage that HSCT can mitigate. This benefit must be balanced against preparative regimen toxicity, graft-versus-host disease, and mortality risk. We review efforts to balance HSCT complications with the promise of cure, and knowledge gaps that warrant further investigation. We highlight the burden of SCD, HSCT risks and benefits, and SCD families' approach to this balance. We emphasize the necessity for information exchange to ensure a joint decision-making process between providers and patients.

Keywords: hematopoietic stem cell transplant; sickle cell disease; transplant eligibility.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Anemia, Sickle Cell / therapy*
  • Child
  • Clinical Decision-Making
  • Clinical Trials as Topic
  • Combined Modality Therapy
  • Female
  • Forecasting
  • Graft vs Host Disease / etiology
  • Graft vs Host Disease / prevention & control
  • Health Services Accessibility
  • Hematopoietic Stem Cell Transplantation* / methods
  • Hematopoietic Stem Cell Transplantation* / statistics & numerical data
  • Hematopoietic Stem Cell Transplantation* / trends
  • Humans
  • Male
  • Patient Selection
  • Professional-Patient Relations
  • Risk
  • Tissue Donors
  • Tissue and Organ Procurement
  • Transplantation Conditioning / adverse effects
  • Transplantation Conditioning / methods
  • Transplantation Conditioning / trends