When are transfusions indicated in the treatment of sickle cell disease (SCD)?

Updated: May 12, 2021
  • Author: Joseph E Maakaron, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Blood transfusions are not needed for the usual anemia or episodes of pain associated with SCD. Urgent replacement of blood is often required for sudden, severe anemia due to acute splenic sequestration, parvovirus B19 infection, or hyperhemolytic crises. Transfusions are helpful in acute chest syndrome, perioperatively, and during pregnancy. Acute red cell exchange transfusion is indicated in the following situations:

  • Acute infarctive stroke
  • Severe acute chest syndrome
  • Multiorgan failure syndromes
  • Right upper quadrant syndrome
  • Priapism that does not resolve after adequate hydration and analgesia

Regular blood transfusions are used for primary and secondary stroke prevention in children with SCD. See Stroke Prevention, below. In addition, Hilliard et al reported that in pediatric patients with frequent pain episodes despite being prescribed hydroxyurea, 1 year of red blood cell transfusion therapy significantly reduced the number of total emergency department visits for pain (6 vs 2.5 pain visits/year, P = 0.005), mean hospitalizations for pain (3.4 vs 0.9 pain admissions/year), and mean hospital days per year for pain crisis (23.5 vs 4.5, P = 0.0001). [75]


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