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Chronic Administration of Hydroxyurea (HU) Benefits Caucasian Patients with Sickle-Beta Thalassemia.
Int J Mol Sci. 2018 Feb 28; 19(3)IJ

Abstract

In sickle cell disease (SCD), hydroxyurea (HU) treatment decreases the number of vaso-occlusive crisis (VOC) and acute chest syndrome (ACS) by increasing fetal hemoglobin (HbF). Data are lacking regarding the frequency of HU dose modification or whether sub-therapeutic doses (<15 mg/kg/day) are beneficial. We reviewed the medical records of 140 patients from 2010 to 2014. The laboratory parameters and SCD complications were compared between the first and last visits based on HU use. Fifty patients (36%) never took HU or suspended HU ("no HU" group). Among patients taking <15 mg/kg/day HU on their first visit, half remained at the same dose, and the other half increased to ≥15 mg/kg/day. Among patients taking ≥15 mg/kg/day, 17% decreased to <15 mg/kg/day, and 83% stayed at ≥15 mg/kg/day. The "no HU" group had fewer episodes of VOC and ACS. Both HU treatment groups had a reduction in both complications (p < 0.0001). This improvement was observed in all SCD phenotypes. The white blood cell (WBC) counts were found to be lower, and HbF increased in both HU groups (p = 0.004, 0.001). The maximal HbF response to HU in HbS/β⁺-thalassemia was 20%, similar to those observed for HbSS (19%) and HbS/β⁰-thalassemia (22%). HbS/β⁺-thalassemia could have a similar disease severity as HbSS or HbS/β⁰-thalassemia. Patients with HbS/β⁰-thalassemia or HbS/β⁺-thalassemia phenotypes responded to HU.

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Authors+Show Affiliations

Di Maggio R
Campus of Haematology Franco and Piera Cutino, AOOR Villa Sofia-V. Cervello, 90142 Palermo, Italy. rdm83@hotmail.it.
Hsieh MM
Molecular and Clinical Hematology Branch, National Institute of Diabetes and Digestive and Kidney Diseases/National Heart, Lung, and Blood Institute, Bethesda, MD 20814, USA. matthewhs@nhlbi.nih.gov.
Zhao X
Office of Clinical Director, National Institute of Diabetes and Digestive and Kidney Diseases, Bethesda, MD 20814, USA. Xiongce.Zhao@fda.hhs.gov.
Calvaruso G
Campus of Haematology Franco and Piera Cutino, AOOR Villa Sofia-V. Cervello, 90142 Palermo, Italy. calvaruso.giuseppina@gmail.com.
Rigano P
Campus of Haematology Franco and Piera Cutino, AOOR Villa Sofia-V. Cervello, 90142 Palermo, Italy. paolorigano@tiscali.it.
Renda D
Campus of Haematology Franco and Piera Cutino, AOOR Villa Sofia-V. Cervello, 90142 Palermo, Italy. dismare@gmail.com.
Tisdale JF
Molecular and Clinical Hematology Branch, National Institute of Diabetes and Digestive and Kidney Diseases/National Heart, Lung, and Blood Institute, Bethesda, MD 20814, USA. johntis@nhlbi.nih.gov.
Maggio A
Campus of Haematology Franco and Piera Cutino, AOOR Villa Sofia-V. Cervello, 90142 Palermo, Italy. md.amaggio@gmail.com.

MeSH

AdolescentAdultAnemia, Sickle CellAntisickling AgentsChildChild, PreschoolErythrocyte IndicesFemaleFollow-Up StudiesHeart Function TestsHumansHydroxyureaInfantLiver Function TestsMaleMiddle AgedPhenotypeTreatment OutcomeYoung Adultbeta-Thalassemia

Pub Type(s)

Journal Article

Language

eng

PubMed ID

29495591

Citation

Di Maggio, Rosario, et al. "Chronic Administration of Hydroxyurea (HU) Benefits Caucasian Patients With Sickle-Beta Thalassemia." International Journal of Molecular Sciences, vol. 19, no. 3, 2018.
Di Maggio R, Hsieh MM, Zhao X, et al. Chronic Administration of Hydroxyurea (HU) Benefits Caucasian Patients with Sickle-Beta Thalassemia. Int J Mol Sci. 2018;19(3).
Di Maggio, R., Hsieh, M. M., Zhao, X., Calvaruso, G., Rigano, P., Renda, D., Tisdale, J. F., & Maggio, A. (2018). Chronic Administration of Hydroxyurea (HU) Benefits Caucasian Patients with Sickle-Beta Thalassemia. International Journal of Molecular Sciences, 19(3). https://doi.org/10.3390/ijms19030681
Di Maggio R, et al. Chronic Administration of Hydroxyurea (HU) Benefits Caucasian Patients With Sickle-Beta Thalassemia. Int J Mol Sci. 2018 Feb 28;19(3) PubMed PMID: 29495591.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Chronic Administration of Hydroxyurea (HU) Benefits Caucasian Patients with Sickle-Beta Thalassemia. AU - Di Maggio,Rosario, AU - Hsieh,Matthew M, AU - Zhao,Xiongce, AU - Calvaruso,Giuseppina, AU - Rigano,Paolo, AU - Renda,Disma, AU - Tisdale,John F, AU - Maggio,Aurelio, Y1 - 2018/02/28/ PY - 2018/01/05/received PY - 2018/02/23/revised PY - 2018/02/26/accepted PY - 2018/3/3/entrez PY - 2018/3/3/pubmed PY - 2018/8/28/medline KW - fetal hemoglobin KW - hydroxyurea KW - sickle beta thalassemia KW - sickle cell disease JF - International journal of molecular sciences JO - Int J Mol Sci VL - 19 IS - 3 N2 - In sickle cell disease (SCD), hydroxyurea (HU) treatment decreases the number of vaso-occlusive crisis (VOC) and acute chest syndrome (ACS) by increasing fetal hemoglobin (HbF). Data are lacking regarding the frequency of HU dose modification or whether sub-therapeutic doses (<15 mg/kg/day) are beneficial. We reviewed the medical records of 140 patients from 2010 to 2014. The laboratory parameters and SCD complications were compared between the first and last visits based on HU use. Fifty patients (36%) never took HU or suspended HU ("no HU" group). Among patients taking <15 mg/kg/day HU on their first visit, half remained at the same dose, and the other half increased to ≥15 mg/kg/day. Among patients taking ≥15 mg/kg/day, 17% decreased to <15 mg/kg/day, and 83% stayed at ≥15 mg/kg/day. The "no HU" group had fewer episodes of VOC and ACS. Both HU treatment groups had a reduction in both complications (p < 0.0001). This improvement was observed in all SCD phenotypes. The white blood cell (WBC) counts were found to be lower, and HbF increased in both HU groups (p = 0.004, 0.001). The maximal HbF response to HU in HbS/β⁺-thalassemia was 20%, similar to those observed for HbSS (19%) and HbS/β⁰-thalassemia (22%). HbS/β⁺-thalassemia could have a similar disease severity as HbSS or HbS/β⁰-thalassemia. Patients with HbS/β⁰-thalassemia or HbS/β⁺-thalassemia phenotypes responded to HU. SN - 1422-0067 UR - https://www.unboundmedicine.com/medline/citation/29495591/Chronic_Administration_of_Hydroxyurea__HU__Benefits_Caucasian_Patients_with_Sickle_Beta_Thalassemia_ DB - PRIME DP - Unbound Medicine ER -