How is anemia treated in patients with sickle cell nephropathy (SCN)?

Updated: Sep 12, 2019
  • Author: Phuong-Thu Pham, MD, FASN; Chief Editor: Vecihi Batuman, MD, FASN  more...
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Answer

Answer

Anemia in patients with SCD is managed differently from anemia due to chronic kidney disease. The recommended hemoglobin (Hb) target should be an Hb concentration of no greater than 10-10.5 g/dL (or a hematocrit of no greater than 30%). In addition, a rise in the hematocrit of greater than 1-2% per week should be avoided. [19, 34] Higher Hb levels and more rapid correction of anemia may precipitate a vasoocclusive crisis. 

Blood transfusions or erythrocyte-stimulating agents (ESA), such as erythropoietin or darbepoetin alfa, may be used to achieve the appropriate hemoglobin concentration. While blood transfusions provide a higher proportion of HbA compared with patients’ own blood, ESA likely does not provide a similar benefit and may be associated with increased vaso-occlusive risk. ESA dosing may be higher in individuals receiving hydroxyurea due to its inherent bone marrow suppressive effect.  However, it has also been suggested that the addition of ESA may allow administration of higher doses of hydroxyurea and improved fetal hemoglobin levels. [35]

For patients presenting with severe anemia and/or hemolytic crisis, appropriate hematology consultation is recommended.


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