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Iron Overload in Sickle Cell Anemia
As transfusion therapy has become more widely used in the treatment of patients with sickle cell disease (SCD), iron overload has become increasingly more common (1). The National Heart, Lung, and Blood Institute (NHLBI) recommends screening for iron overload with regular serum ferritin testing at the onset of therapy (2).
Increasing use of transfusion therapy
Results of the Stroke Prevention in Sickle Cell Anemia (STOP) trial showing 92% stroke risk reduction in at-risk children who received transfusion therapy (3) represented a turning point in the use of transfusion therapy in patients with SCD. The trend toward increasing use of blood transfusions was further supported by the STOP 2 trial, which was halted after data showed that high-risk patients' chances of remaining event-free decreased by nearly half within 12 months after transfusion therapy was discontinued (4). NHLBI guidelines interpret these results as potentially supporting the use of transfusion therapy in adults as well as children (2).
Transfusion therapy can prevent cerebrovascular events in high-risk SCD patientsTransfusion therapy can prevent cerebrovascular events in high-risk SCD patients
A Kaplan-Meier estimate of probability of no end-point events decreased rapidly after transfusion therapy was discontinued. "Events" were either cardiovascular events or abnormal TCD readings. Adapted with permission from Adams, et al (4).
Indications for transfusion therapy in SCD
According to National Heart, Lung, and Blood Institute guidelines, chronic transfusion therapy may be indicated for a variety of reasons, including stroke prevention (in high-risk adults as well as in children), chronic debilitating pain, pulmonary hypertension, and anemia associated with chronic renal failure [ref NHLBI]. Episodic transfusions may be indicated for the management of severe anemia, for severe illnesses such as acute chest syndrome, or in preparation for surgery (2).
Iron overload and mortality in SCD
As transfusion therapy has become more widely used in the treatment of patients with SCD, iron overload has become more common (1).
Iron overload is associated with increased mortality in SCDIron overload is associated with increased mortality in SCD
Mortality over a 12-year period in patients with and without iron overload. Adapted with permission from Ballas, et al [Ballas, 2001].
NHLBI recommendations for iron overload screening
The National Heart, Lung, and Blood Institute (NHLBI) recommends screening for iron overload with regular serum ferritin testing at the onset of transfusions, and states that overload is likely to be detectable after 20 transfusions (2).
Learn more about the NHLBI recommendations
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Transfusional Iron Overload
Sickle Cell Anemia
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Serum Ferritin
>1000 mcg/L in SCD
NHLBI guidelines recommend monitoring your iron levels for iron overload when serum ferritin is >1000 mcg/L.
Learn moreReferences
- (1) Reed WF, Vichinsky EP, Transfusion practice for patients with sickle cell disease. Curr Opin Hematol. 1999;6(6):432-6.
- (2) National Institutes of Health, National Heart, Lung, and Blood Institute: The Management of Sickle Cell Disease. 2002, Bethesda, MD: NIH.
- (3) Adams RJ, McKie VC, Hsu L, et al, Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography. N Engl J Med. 1998;339(1):5-11.
- (4) Adams RJ, Brambilla D. Discontinuing prophylactic transfusions used to prevent stroke in sickle cell disease. N Engl J Med. 2005;353(26):2769-78.