FRCPath Haematology Part 2: Transfusion Blood Provision: Special Requirements Sickle Cell Disease
  • Patients with sickle cell disease have a high incidence of alloantibody formation.
  • Severe haemolytic transfusion reactions are not uncommon.
  • Transfusions in SCD requires careful consideration of both the haemoglobin concentration and the percentage of sickle haemoglobin in order to preserve maximal tissue oxygen delivery, without increasing the blood viscosity.
  • Prior to transfusion, patients should undergo extended red cell phenotyping, which should include: C, c, E, e, K, k, Jka, Jkb, Fya, Fyb, S and s antigens. 
    • If a patient is S- and s-, then U typing should be performed.
    • Patients who are U negative should receive U negative blood to avoid formation of anti-U.
  • Blood provided for SCD patients must be (if possible):
    • ABO compatible
    • Be matched for Rh (D, C, c, E and e) and Kell antigens at a minimum.
    • If there are any alloantibodies, the blood must be negative for the corresponding antigen.
    • Fresh red cells to enhance red cell survival:
      • Less than 10 days old for simple transfusion.
      • Less than 7 days old for exchange transfusion.
    • HbS negative (via the sickle solubility test).
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