What are the ASH guidelines on the treatment of chronic kidney disease in patients with sickle cell disease (SCD)?

Updated: May 12, 2021
  • Author: Joseph E Maakaron, MD; Chief Editor: Emmanuel C Besa, MD  more...
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In children and adults with SCD and worsening anemia associated with chronic kidney disease (CKD), the ASH guideline panel suggests combination therapy with hydroxyurea and erythropoiesis-stimulating agents. This recommendation is based on evidence available only from patients with hemoglobin SS or S/β0 thalassemia, for whom erythropoiesis-stimulating-agent dosing in the studies reviewed was higher than that typically used in the general population.

For patients with SCD and CKD who are already on steady-state hydroxyurea, starting erythropoiesis-stimulating agents is appropriate when a simultaneous drop occurs in the hemoglobin concentration and absolute reticulocyte count. Optimizing adherence to hydroxyurea therapy while on erythropoiesis-stimulating agents may help maximize fetal hemoglobin responses for patients treated with combination therapy.

For patients with SCD undergoing treatment with erythropoiesis-stimulating agents, a conservative hemoglobin threshold is advised above which treatment should be decreased or held. The ASH guideline panel advises not exceeding a hemoglobin threshold of 10 g/dL (hematocrit of 30%) to reduce the risk of vaso-occlusion–related complications, stroke, and venous thromboembolism.

For children and adults with SCD and advanced CKD or end-stage renal disease, the ASH guideline panel suggests referral for renal transplantation. Judicious use of corticosteroids as part of the posttransplant immunosuppression regimen is advised given the potential relationship between steroid exposure and vaso-occlusive pain for patients with SCD.

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