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The effect of prolonged administration of hydroxyurea on morbidity and mortality in adult patients with sickle cell syndromes: results of a 17-year, single-center trial (LaSHS).
Blood 2010; 115(12):2354-63Blood

Abstract

The aim of this prospective study was to evaluate the long-term efficacy and safety of hydroxyurea (HU) in patients with sickle cell disease (SCD). Thirty-four patients with sickle cell anemia (hemoglobin S [HbS]/HbS), 131 with HbS/beta(0)-thal, and 165 with HbS/beta(+)-thal participated in this trial. HU was administered to 131 patients, whereas 199 patients were conventionally treated. The median follow-up period was 8 years for HU patients and 5 years for non-HU patients. HU produced a dramatic reduction in the frequency of severe painful crises, transfusion requirements, hospital admissions, and incidence of acute chest syndrome. The probability of 10-year survival was 86% and 65% for HU and non-HU patients, respectively (P = .001), although HU patients had more severe forms of SCD. The 10-year probability of survival for HbS/HbS, HbS/beta (0)-thal, and HbS/IVSI-110 patients was 100%, 87%, and 82%, respectively, for HU patients and 10%, 54%, and 66%, for non-HU patients. The multivariate analysis showed that fetal hemoglobin values at baseline and percentage change of lactate dehydrogenase between baseline and 6 months were independently predicted for survival in the HU group. These results highlight the beneficial effect of HU, which seems to modify the natural history of SCD and raise the issue of expanding its use in all SCD patients.

Authors+Show Affiliations

Thalassemia Center, Laikon General Hospital, GR-11526 Athens, Greece. ersi_voskaridou@yahoo.comNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Clinical Trial, Phase II
Journal Article

Language

eng

PubMed ID

19903897

Citation

Voskaridou, Ersi, et al. "The Effect of Prolonged Administration of Hydroxyurea On Morbidity and Mortality in Adult Patients With Sickle Cell Syndromes: Results of a 17-year, Single-center Trial (LaSHS)." Blood, vol. 115, no. 12, 2010, pp. 2354-63.
Voskaridou E, Christoulas D, Bilalis A, et al. The effect of prolonged administration of hydroxyurea on morbidity and mortality in adult patients with sickle cell syndromes: results of a 17-year, single-center trial (LaSHS). Blood. 2010;115(12):2354-63.
Voskaridou, E., Christoulas, D., Bilalis, A., Plata, E., Varvagiannis, K., Stamatopoulos, G., ... Terpos, E. (2010). The effect of prolonged administration of hydroxyurea on morbidity and mortality in adult patients with sickle cell syndromes: results of a 17-year, single-center trial (LaSHS). Blood, 115(12), pp. 2354-63. doi:10.1182/blood-2009-05-221333.
Voskaridou E, et al. The Effect of Prolonged Administration of Hydroxyurea On Morbidity and Mortality in Adult Patients With Sickle Cell Syndromes: Results of a 17-year, Single-center Trial (LaSHS). Blood. 2010 Mar 25;115(12):2354-63. PubMed PMID: 19903897.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - The effect of prolonged administration of hydroxyurea on morbidity and mortality in adult patients with sickle cell syndromes: results of a 17-year, single-center trial (LaSHS). AU - Voskaridou,Ersi, AU - Christoulas,Dimitrios, AU - Bilalis,Antonios, AU - Plata,Eleni, AU - Varvagiannis,Konstantinos, AU - Stamatopoulos,George, AU - Sinopoulou,Klio, AU - Balassopoulou,Aggeliki, AU - Loukopoulos,Dimitris, AU - Terpos,Evangelos, Y1 - 2009/11/10/ PY - 2009/11/12/entrez PY - 2009/11/12/pubmed PY - 2010/4/14/medline SP - 2354 EP - 63 JF - Blood JO - Blood VL - 115 IS - 12 N2 - The aim of this prospective study was to evaluate the long-term efficacy and safety of hydroxyurea (HU) in patients with sickle cell disease (SCD). Thirty-four patients with sickle cell anemia (hemoglobin S [HbS]/HbS), 131 with HbS/beta(0)-thal, and 165 with HbS/beta(+)-thal participated in this trial. HU was administered to 131 patients, whereas 199 patients were conventionally treated. The median follow-up period was 8 years for HU patients and 5 years for non-HU patients. HU produced a dramatic reduction in the frequency of severe painful crises, transfusion requirements, hospital admissions, and incidence of acute chest syndrome. The probability of 10-year survival was 86% and 65% for HU and non-HU patients, respectively (P = .001), although HU patients had more severe forms of SCD. The 10-year probability of survival for HbS/HbS, HbS/beta (0)-thal, and HbS/IVSI-110 patients was 100%, 87%, and 82%, respectively, for HU patients and 10%, 54%, and 66%, for non-HU patients. The multivariate analysis showed that fetal hemoglobin values at baseline and percentage change of lactate dehydrogenase between baseline and 6 months were independently predicted for survival in the HU group. These results highlight the beneficial effect of HU, which seems to modify the natural history of SCD and raise the issue of expanding its use in all SCD patients. SN - 1528-0020 UR - https://www.unboundmedicine.com/medline/citation/19903897/full_citation L2 - http://www.bloodjournal.org/cgi/pmidlookup?view=long&pmid=19903897 DB - PRIME DP - Unbound Medicine ER -