John Libbey Eurotext

Médecine thérapeutique / Pédiatrie


Features of blood transfusion in children with sickle cell disease Volume 20, issue 4, Octobre-Novembre-Décembre 2017


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1 UMR_S1134, Inserm, Université Paris Diderot, Sorbonne Paris Cité, Institut National de la Transfusion Sanguine, Paris, F-75015
2 Laboratoire d’Excellence GR-Ex
3 AP-HP, Hôpital Armand Trousseau, Service de Pédiatrie, 26 avenue du Dr Arnold Netter, F-75012 Paris
4 Institut National de la Transfusion Sanguine, Département Centre National de Référence pour les Groupes Sanguins, 20 rue Bouvier, BP 79, 75011 Paris, France
* Tirés à part
  • Key words: child, sickle cell disease, transfusion, alloimmunisation, delayed haemolytic transfusion reaction, blood groups
  • DOI : 10.1684/mtp.2018.0659
  • Page(s) : 254-64
  • Published in: 2017

The therapeutic management of sickle cell disease is based on several strategies, in which red blood cell transfusion plays an essential role in acute complications such as vaso-occlusive crisis, acute chest syndrome and stroke. However, it is important to weigh the benefit/risk before transfusion in children with sickle cell disease and not to rely solely on the value of hemoglobin. Indeed, it is important to remember that a negative antibody screen as well as a negative serological crossmatch test do not totally eliminate alloimmunisation or the risk of post-transfusion haemolysis. Sickle cell patients demonstrate multiple immuno-haematological features: variant phenotypes (especially in the Rh and MNS systems), rare blood groups, allo- and auto-immunisation favored because of the inflammatory state. The presence of an alloimmunisation can lead to a significant delay to obtain compatible red blood cell units, a supply difficulty or even a genuine blood transfusion deadlock. About 30 % of the requests for rare blood in France concerns sickle cell patients. Any vaso-occlusive crisis occurring within 3 to 15 days after a transfusion should be suspected to be a delayed haemolytic post-transfusion reaction; whenever necessary, the child should be referred to a reference center and any further transfusions should be avoided. The so-called hyperhaemolysis syndrome, corresponding to a major delayed haemolysis with concomitant destruction of autologous red blood cells, constitutes a major complication of the transfusion and may be potentially fatal. It is essential to educate patients and physicians on the recognition of the clinical signs of delayed haemolytic post-transfusion reactions, in order to rapidly implement measures to limit their immediate effects and avoid their occurrence in case of future transfusion.