title: Erythropoietic activity in patients with sickle cell anaemia before and after treatment with hydroxyurea.
abstract: In this project we have prospectively studied the erythropoietic activity in patients with sickle cell anaemia (SS) before and after treatment with hydroxyurea (HU). Some of the patients were enrolled in a double-blind placebo controlled trial of HU in patients with SS and others were enrolled in an open label study. Determinants of erythropoietic activity included the reticulocyte count, red blood cell (RBC) survival by the 51Cr method, plasma 59Fe clearance, plasma iron turnover (PIT), erythron transferrin uptake (ETU), RBC production/destruction rate, and RBC Fe utilization. Therapy with HU increased the mean corpuscular volume (MCV), haemoglobin (Hb)F, RBC survival and t1/2 59Fe clearance; it decreased the reticulocyte count, the white blood cell (WBC) count, ETU, and the PIT. Most of the changes in parameters of erythropoiesis could be explained by the increase in 51Cr RBC survival after therapy with HU. Together the data showed that in selected patients the net effect of HU on Hb level was a function of the difference between the suppressive effect of HU (decreased RBC production) and the increase in RBC survival. In the majority of patients who responded to HU, there was a preferential effect on RBC survival.
authors.affiliation: Cardeza Foundation for Hematologic Research, Department of Medicine, Jefferson Medical College, Philadelphia, Pennsylvania, USA.
authors.forename: S K
authors.initials: SK
authors.lastname: Ballas
authors.forename: M J
authors.initials: MJ
authors.lastname: Marcolina
authors.forename: G J
authors.initials: GJ
authors.lastname: Dover
authors.forename: F B
authors.initials: FB
authors.lastname: Barton
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood / Q000188:drug therapy* / Q000473:pathology; D000986:Antisickling Agents / Q000627:therapeutic use*; D002470:Cell Survival; D004311:Double-Blind Method; D004912:Erythrocytes / Q000473:pathology; D004920:Erythropoiesis / Q000187:drug effects*; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use*; D007501:Iron / Q000378:metabolism
chemical_list: D000986:Antisickling Agents; D007501:Iron; D006918:Hydroxyurea