Erythropoietin and Sickle Cell Disease

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Heard an interesting presentation at our Renal Grand Rounds today regarding a young patient with end-stage renal disease secondary to sickle cell disease who is continually admitted for sickle cell crises.

The question arose whether or not erythropoietin therapy would be beneficial in the treatment of his anemia. If the EPO stimulates predominantly HgbS production, one could make the case that it may actually induce sickling. However, there is some data (Little et al, Heamatologica 2006) that when given in conjunction with hydroxyurea, erythropoietin actually increases HgbF production and is safe in the setting of sickle cell disease.

Sickle cell disease has a variety of renal manifestations–largely as the result of vaso-occlusive phenomena–which includes secondary FSGS, hypertension, chronic hematuria, renal infarction and papillary necrosis, nephrogenic diabetes insipidus, Type I RTA, and occasionally ESRD.

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