Transfusing children with hemoglobinopathies

Transfus Clin Biol. 2019 Sep;26(3):147-149. doi: 10.1016/j.tracli.2019.06.323. Epub 2019 Jun 29.

Abstract

Thalassemia and sickle cell disease (SCD) are among the most common inherited diseases worldwide. Red blood cell transfusion is a cornerstone of their treatment, but its indications have significantly changed over the past years. New therapies are emerging in both syndromes: among them, hematopoietic stem cell transplantation is now routinely proposed, and gene therapy has shown promising preliminary results.

Keywords: Drépanocytose; Gene therapy; Greffe de cellules souches hématopoïétiques; Hematopoietic stem cell transplantation; Hydroxyurea; Hydroxyurée; Red blood cell transfusion; Sickle cell disease; Thalassemia; Thalassémie; Thérapie génique; Transfusion érythrocytaire.

Publication types

  • Review

MeSH terms

  • Allografts
  • Anemia, Sickle Cell / drug therapy
  • Anemia, Sickle Cell / therapy
  • Child
  • Emergencies
  • Erythrocyte Transfusion*
  • Exchange Transfusion, Whole Blood
  • Genetic Therapy
  • Hematopoietic Stem Cell Transplantation
  • Hemoglobinopathies / therapy*
  • Humans
  • Hydroxyurea / therapeutic use
  • Multiple Organ Failure / therapy
  • Thalassemia / therapy

Substances

  • Hydroxyurea