Abstract
The suppression of erythropoiesis by Hydroxyurea (HU) therapy is associated with increase in mean corpuscular volume, in addition to the increase in Hb F. Monitoring the mean corpuscular volume values and the presence of macrocytosis are effective tools of adherence to the treatment with HU in patients with sickle cell anemia. The aim of this study is to monitor the mean corpuscular volume values after starting treatment with HU to determine if macrocytosis can be used as a surrogate marker of compliance with therapy. We conducted a prospective cohort study over one year with measurements of blood counts and mean corpuscular volume after starting therapy with HU in 95 patients with sickle cell anemia who were regularly followed in our ambulatory outpatient unit. In one-year of successful use of HU the mean value of the mean corpuscular volume increased significantly. The Andersen and Gill model demonstrated that the increase of one unit of MCV implies a 5% reduction in the risk of visiting the emergency room. Monitoring mean corpuscular volume values after prescribing HU alerts the provider of noncompliance in order to counsel the patient in question for better adherence to the use of HU that could improve the quality of care and to reduce morbidity and the frequency of acute pain crises and associated healthcare costs.
Highlights
Sickle cell anemia (SS) is an inherited chronic inflammatory and degenerative disease in which hemoglobin S (Hb S) is produced
We evaluated 95 patients with Sickle Cell Anemia (SS) with no coexistent alpha or beta thalassemia who were started on HU and followed regularly in the outpatient unit, from February 01, 2010 to February 01, 2011
Three models were considered: the first model used gender, age, the initial Mean Corpuscular Volume (MCV) and the final MCV as independent variables; the second model used gender, age and the value obtained by the difference between initial MCV and final MCV and the third model used gender, age and the dichotomization of this difference (1 if final MCV is less than initial MCV, 0 if otherwise)
Summary
Sickle cell anemia (SS) is an inherited chronic inflammatory and degenerative disease in which hemoglobin S (Hb S) is produced. In its severe form, the disease is characterized by recurrent acute painful crises, severe anemia and organ damage. The recurrent painful episodes often require treatment in the emergency room or the hospital interfering with the quality of life of affected patients [3]. These complications of SS usually manifest themselves around the age of 6 month when the synthesis of the sickle β globin chain reaches adult values and continue through the life span of the patient with progressive severity [4,5,6]
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