Thursday, October 09, 2014

Hydroxyurea and HbF in sickle cell disease

Steinberg MH et al. Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia.    Risks and benefits up to 9 years of treatment. JAMA 1003; 289: 1645-51.  Weiner DL, et al. editorial: hydroxyurea and sickle cell disease. A chance for every patient.

HbF is inversely related to mortality in SCD, and can be increased with hydroxyurea.  Also cite Multicenter Study of hydroxyurea (MSH) in SCA (NEJM 1995; 332: 1317-1322).

299 SCA patients were randomized to hydroxyurea or placebo, morbidity was reduced by half, with no difference in mortality or stroke.  There was an open label extension of the trial on 233 patients (both arms) receiving hydroxyurea for 9 years (up to).  In this part of the study, mortality was reduced forty percent for patients receiving hydroxyurea (patient option to take or not).  Cumulative mortality corresponded with HBF levels esp .5 g/dL or higher. 

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