Choosing Wisely:

Don’t routinely transfuse patients with sickle cell disease for chronic anemia or uncomplicated pain crisis without an appropriate clinical indication.

Rationale and Comments: Patients with sickle cell disease are especially vulnerable to potential harms from unnecessary red blood cell transfusion. In particular, they experience an increased risk of alloimmunization to minor blood group antigens and a high risk of iron overload from repeated transfusions. Patients with the most severe genotypes of sickle cell disease with baseline hemoglobin values in the 7 to 10 g/dL range can usually tolerate further temporary reductions in Hb without developing symptoms of anemia. Many patients with sickle cell disease receive intravenous fluids to improve hydration when hospitalized for management of pain crisis, which may contribute to a decrease in hemoglobin by 1 to 2 g/dL. Routine administration of red blood cells in this setting should be avoided. Moreover, there is no evidence that transfusion reduces pain due to vaso-occlusive crises. For a discussion of when transfusion is indicated in sickle cell disease, readers are referred to recent evidence-based guidelines from the National Heart, Lung, and Blood Institute (see references).
Sponsoring Organizations:
  • American Society of Hematology
  • Sources:
  • National Heart, Lung and Blood Institute guidelines
  • Disciplines:
  • Hematologic
  • References: • Evidence-based management of sickle cell disease: expert panel report, 2014. Washington, DC: National Institutes of Health, National Heart, Lung and Blood Institute; 2014:161.
    • Blood transfusion guideline. Dutch Institute for Healthcare Improvement CBO; 2011:402.

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