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Effects of hydroxyurea on hemoglobin F and water content in the red blood cells of dogs and of patients with sickle cell anemia.
Blood 1991; 78(1):212-6Blood

Abstract

A rationale for clinical trials of hydroxyurea (HU) treatment in sickle cell disease is that the agent increases red blood cell (RBC) fetal hemoglobin content. However, an additional effect of HU is to raise the mean corpuscular volume (MCV). To investigate the action of HU in a species that makes no electrophoretically distinguishable fetal hemoglobin, we treated dogs with the drug and compared their response to that of five patients with sickle cell anemia. Both dogs and patients had an increase in MCV, but the effect of HU treatment on the mean corpuscular hemoglobin concentration (MCHC), density, and water content of the RBCs differed in the two species. The dog RBCs became low in MCHC, high in ion and water content, and low in mean density. Thus, HU can raise MCV and lower MCHC without influencing fetal hemoglobin synthesis. A different pattern was seen in the sickle cell patients during HU treatment. Although the MCV of their RBCs increased, there was no change in MCHC, ion content, or mean density. A notable change in the sickle cell patients' blood was that two subpopulations of cells were nearly eliminated during HU treatment; the hypodense reticulocyte fraction and the hyperdense fraction that contains irreversibly sickled cells. These findings lead us to suggest that trials of HU in sickle cell disease must recognize the possibility that any beneficial effect of this agent might be due not only to an increase in hemoglobin F alone, but perhaps also to the associated increase in MCV or the altered RBC density profile.

Authors+Show Affiliations

Department of Medicine, University of North Carolina at Chapel Hill 27599-7600.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

1712641

Citation

Orringer, E P., et al. "Effects of Hydroxyurea On Hemoglobin F and Water Content in the Red Blood Cells of Dogs and of Patients With Sickle Cell Anemia." Blood, vol. 78, no. 1, 1991, pp. 212-6.
Orringer EP, Blythe DS, Johnson AE, et al. Effects of hydroxyurea on hemoglobin F and water content in the red blood cells of dogs and of patients with sickle cell anemia. Blood. 1991;78(1):212-6.
Orringer, E. P., Blythe, D. S., Johnson, A. E., Phillips, G., Dover, G. J., & Parker, J. C. (1991). Effects of hydroxyurea on hemoglobin F and water content in the red blood cells of dogs and of patients with sickle cell anemia. Blood, 78(1), pp. 212-6.
Orringer EP, et al. Effects of Hydroxyurea On Hemoglobin F and Water Content in the Red Blood Cells of Dogs and of Patients With Sickle Cell Anemia. Blood. 1991 Jul 1;78(1):212-6. PubMed PMID: 1712641.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Effects of hydroxyurea on hemoglobin F and water content in the red blood cells of dogs and of patients with sickle cell anemia. AU - Orringer,E P, AU - Blythe,D S, AU - Johnson,A E, AU - Phillips,G,Jr AU - Dover,G J, AU - Parker,J C, PY - 1991/7/11/pubmed PY - 2001/3/28/medline PY - 1991/7/11/entrez SP - 212 EP - 6 JF - Blood JO - Blood VL - 78 IS - 1 N2 - A rationale for clinical trials of hydroxyurea (HU) treatment in sickle cell disease is that the agent increases red blood cell (RBC) fetal hemoglobin content. However, an additional effect of HU is to raise the mean corpuscular volume (MCV). To investigate the action of HU in a species that makes no electrophoretically distinguishable fetal hemoglobin, we treated dogs with the drug and compared their response to that of five patients with sickle cell anemia. Both dogs and patients had an increase in MCV, but the effect of HU treatment on the mean corpuscular hemoglobin concentration (MCHC), density, and water content of the RBCs differed in the two species. The dog RBCs became low in MCHC, high in ion and water content, and low in mean density. Thus, HU can raise MCV and lower MCHC without influencing fetal hemoglobin synthesis. A different pattern was seen in the sickle cell patients during HU treatment. Although the MCV of their RBCs increased, there was no change in MCHC, ion content, or mean density. A notable change in the sickle cell patients' blood was that two subpopulations of cells were nearly eliminated during HU treatment; the hypodense reticulocyte fraction and the hyperdense fraction that contains irreversibly sickled cells. These findings lead us to suggest that trials of HU in sickle cell disease must recognize the possibility that any beneficial effect of this agent might be due not only to an increase in hemoglobin F alone, but perhaps also to the associated increase in MCV or the altered RBC density profile. SN - 0006-4971 UR - https://www.unboundmedicine.com/medline/citation/1712641/Effects_of_hydroxyurea_on_hemoglobin_F_and_water_content_in_the_red_blood_cells_of_dogs_and_of_patients_with_sickle_cell_anemia_ L2 - http://www.bloodjournal.org/cgi/pmidlookup?view=long&pmid=1712641 DB - PRIME DP - Unbound Medicine ER -