Type your tag names separated by a space and hit enter

Reduction of the clinical severity of sickle cell/beta-thalassemia with hydroxyurea: the experience of a single center in Greece.

Abstract

The use of hydroxyurea for the prevention of sickle cell crises in patients with homozygous HbS disease is now well established. The beneficial effects of this compound stem from (a) selective enrichment of red cells containing an increased amount of fetal hemoglobin, which inhibits HbS polymerization, and (b) a decrease of leukocytes, platelets, and reticulocytes, which significantly limits their adherence to the vascular wall. We report the results of a clinical trial of hydroxyurea on 55 Greek-origin patients with sickle cell/beta-thalassemia and 14 patients with homozygous HbS disease who have been treated with hydroxyurea for several years. Such patients have a higher probability to benefit from hydroxyurea therapy, since in addition to its antisickling effect, the increase of gamma-chain synthesis is expected to diminish the deleterious effects of the unbound alpha-globin chains. Selection of patients and monitoring throughout the whole trial were done by the same clinicians. Quantitative expression of the clinical condition was done using a system scoring several outcome parameters. For a period of 52 months prior to starting treatment, the total score of severity for 59 evaluable patients was 1182 points (3068 patient-weeks), while for the 12,018 patient-weeks of the trial this parameter fell to only 82 points. Other observations of interest include the significant improvement of a group of patients with hepatic cholestasis, the development of leg ulcers possibly related to the treatment, and the dramatic increase of hemoglobin F, often in association with an increase of the total hemoglobin levels as a result of decreased hemolysis.

Links

  • Publisher Full Text
  • Authors+Show Affiliations

    ,

    First Department of Medicine, University of Athens, 115 27 Athens, Greece. dloukop@otenet.gr

    , , , ,

    Source

    Blood cells, molecules & diseases 26:5 2000 Oct pg 453-66

    MeSH

    Acute Disease
    Adolescent
    Adult
    Anemia, Sickle Cell
    Antisickling Agents
    Erythrocyte Count
    Erythrocyte Indices
    Erythrocytes
    Erythropoietin
    Female
    Fetal Hemoglobin
    Greece
    Hemoglobins
    Humans
    Hydroxyurea
    Leg Ulcer
    Male
    Middle Aged
    Pancreatitis
    Patient Compliance
    Patient Selection
    Receptors, Transferrin
    Severity of Illness Index
    beta-Thalassemia

    Pub Type(s)

    Clinical Trial
    Journal Article
    Research Support, Non-U.S. Gov't

    Language

    eng

    PubMed ID

    11112383

    Citation

    Loukopoulos, D, et al. "Reduction of the Clinical Severity of Sickle Cell/beta-thalassemia With Hydroxyurea: the Experience of a Single Center in Greece." Blood Cells, Molecules & Diseases, vol. 26, no. 5, 2000, pp. 453-66.
    Loukopoulos D, Voskaridou E, Kalotychou V, et al. Reduction of the clinical severity of sickle cell/beta-thalassemia with hydroxyurea: the experience of a single center in Greece. Blood Cells Mol Dis. 2000;26(5):453-66.
    Loukopoulos, D., Voskaridou, E., Kalotychou, V., Schina, M., Loutradi, A., & Theodoropoulos, I. (2000). Reduction of the clinical severity of sickle cell/beta-thalassemia with hydroxyurea: the experience of a single center in Greece. Blood Cells, Molecules & Diseases, 26(5), pp. 453-66.
    Loukopoulos D, et al. Reduction of the Clinical Severity of Sickle Cell/beta-thalassemia With Hydroxyurea: the Experience of a Single Center in Greece. Blood Cells Mol Dis. 2000;26(5):453-66. PubMed PMID: 11112383.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - Reduction of the clinical severity of sickle cell/beta-thalassemia with hydroxyurea: the experience of a single center in Greece. AU - Loukopoulos,D, AU - Voskaridou,E, AU - Kalotychou,V, AU - Schina,M, AU - Loutradi,A, AU - Theodoropoulos,I, PY - 2000/12/9/pubmed PY - 2001/2/28/medline PY - 2000/12/9/entrez SP - 453 EP - 66 JF - Blood cells, molecules & diseases JO - Blood Cells Mol. Dis. VL - 26 IS - 5 N2 - The use of hydroxyurea for the prevention of sickle cell crises in patients with homozygous HbS disease is now well established. The beneficial effects of this compound stem from (a) selective enrichment of red cells containing an increased amount of fetal hemoglobin, which inhibits HbS polymerization, and (b) a decrease of leukocytes, platelets, and reticulocytes, which significantly limits their adherence to the vascular wall. We report the results of a clinical trial of hydroxyurea on 55 Greek-origin patients with sickle cell/beta-thalassemia and 14 patients with homozygous HbS disease who have been treated with hydroxyurea for several years. Such patients have a higher probability to benefit from hydroxyurea therapy, since in addition to its antisickling effect, the increase of gamma-chain synthesis is expected to diminish the deleterious effects of the unbound alpha-globin chains. Selection of patients and monitoring throughout the whole trial were done by the same clinicians. Quantitative expression of the clinical condition was done using a system scoring several outcome parameters. For a period of 52 months prior to starting treatment, the total score of severity for 59 evaluable patients was 1182 points (3068 patient-weeks), while for the 12,018 patient-weeks of the trial this parameter fell to only 82 points. Other observations of interest include the significant improvement of a group of patients with hepatic cholestasis, the development of leg ulcers possibly related to the treatment, and the dramatic increase of hemoglobin F, often in association with an increase of the total hemoglobin levels as a result of decreased hemolysis. SN - 1079-9796 UR - https://www.unboundmedicine.com/medline/citation/11112383/Reduction_of_the_clinical_severity_of_sickle_cell/beta_thalassemia_with_hydroxyurea:_the_experience_of_a_single_center_in_Greece_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1079-9796(00)90328-0 DB - PRIME DP - Unbound Medicine ER -