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Sickle Cell Disease: Blood Transfusion Therapy

Blood transfusion therapy is the main treatment approach for sickle cell disease (SCD).1,2 It provides effective treatment for many complications by increasing hemoglobin (Hb) levels (and thus the oxygen-carrying capacity of blood) and decreasing the proportion of sickled red blood cells (RBCs) (Table).3,4

Table. Indications for transfusion therapy in SCD3,4

Type of transfusion therapy Indication
Acute/episodic
  • Anemia
  • Splenic sequestration
  • Severe or long-lasting aplastic crises
  • Stroke
  • Acute chest syndrome
  • Multiple-organ failure syndrome
  • Pre-operative (in select cases)
  • Malaria-associated severe haemolytic anemia with impending cardiac decompensation
Chronic
  • Heart failure
  • Prophylaxis against recurrent stroke
  • Stroke prevention when TCD ultrasonography velocities are abnormal
  • Chronic pulmonary hypertension (unresponsive to other approaches)
  • Severe recurrent vaso-occlusive crises
  • Previous splenic sequestration in a child aged 2-3 years (in anticipation of later splenectomy)
  • Chronic pain

SCD = sickle cell disease; TCD = transcranial Doppler.

Goals of Transfusion Therapy

Treatment goals include reducing the sickle Hb (HbS) concentration to < 30% of total Hb, while ensuring that total Hb does not exceed 11 g/dL (or packed cell volume of 35%), a level that may cause complications due to increased viscosity.5

Did You Know...?

The overall transfusion burden is generally less in sickle cell disease patients than in thalassemia patients, who require lifelong transfusion therapy.

Historically, transfusion has been used intermittently in patients with SCD for the treatment of acute events. The overall transfusion burden is generally less in SCD patients than in thalassemia patients, who require lifelong transfusion therapy.

The value of chronic transfusions in potentially reducing hospitalization rates due to vaso-occlusive crises, acute chest syndrome, infection, and stroke is increasingly recognized.6 In addition, as life expectancy continues to increase, more patients are likely to receive long-term transfusion therapy.7

Learn about consequences of regular blood transfusion >

References

  1. Ataga KI. Hematology Am Soc Hematol Educ Program. 2009:54-61.
  2. Buchanan G, et al. Biol Blood Marrow Transplant. 2010;16 Suppl:S64-7.
  3. Stuart MJ, Nagel RL. Lancet. 2004;364:1343-60.
  4. Vichinsky E. Semin Hematol. 2001;38 Suppl 1:2-4.
  5. Schmalzer EA, et al. Transfusion. 1987;27:228-33.
  6. Styles AL, Vichinsky E. J Pediatr. 1994;125:909-11.
  7. Claster S, Vichinsky EP. BMJ. 2003;327:1151-5.

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