Tags

Type your tag names separated by a space and hit enter

An age dependent response to hydroxyurea in pediatric sickle cell anemia patients with alpha thalassemia trait.
Blood Cells Mol Dis. 2017 07; 66:19-23.BC

Abstract

Hydroxyurea (HU) is a key drug therapy for individuals with sickle cell anemia (SCA), yet its clinical and hematologic responses can be variable. Various studies have reported the role of α-thalassemia as one of the most prevalent heritable traits that may modify HU response. We provide data from 62 pediatric and adolescent patients with SCA, 26 with co-inherited α-thalassemia trait. Our data suggest that altered hematologic and clinical responses to HU therapy are noted in adolescent SCA individuals with co-inherited α-thalassemia trait. Adolescent patients who co-inherited α-thalassemia trait had a greater reduction in vaso-occlusive episodes compared to those without α-thalassemia, despite a less robust fetal hemoglobin induction as well as a lower maximum HU dose. This clinical improvement was associated with a lower MCH and higher RBC count. Responses to HU in younger SCA children (ages 5-11years) with co-inherited α-thalassemia trait, compared to those without α-thalassemia trait, did not show any difference in number vaso-occlusive episodes, fetal hemoglobin induction and change in MCH and RBC count.

Links

Publisher Full Text

Authors+Show Affiliations

Figueiredo L
Children's Hospital at Montefiore, Department of Pediatrics, Division of Hematology/Oncology, Bronx, NY, United States; Department of Pediatrics, Albert Einstein College of Medicine, Bronx, NY, United States. Electronic address: lfigueir@montefiore.org.
Morrone K
Children's Hospital at Montefiore, Department of Pediatrics, Division of Hematology/Oncology, Bronx, NY, United States; Department of Pediatrics, Albert Einstein College of Medicine, Bronx, NY, United States.
Wei C
Department of Pediatrics, Albert Einstein College of Medicine, Bronx, NY, United States.
Ireland K
Children's Hospital at Montefiore, Department of Pediatrics, Division of Hematology/Oncology, Bronx, NY, United States.
Cohen HW
Dept. of Epidemiology and Population Health, Albert Einstein College of Medicine, Bronx, NY, United States.
Driscoll C
Children's Hospital at Montefiore, Department of Pediatrics, Division of Hematology/Oncology, Bronx, NY, United States; Department of Pediatrics, Albert Einstein College of Medicine, Bronx, NY, United States.
Manwani D
Children's Hospital at Montefiore, Department of Pediatrics, Division of Hematology/Oncology, Bronx, NY, United States; Department of Pediatrics, Albert Einstein College of Medicine, Bronx, NY, United States.

MeSH

AdolescentAge FactorsAnemia, Sickle CellArterial Occlusive DiseasesChildChild, PreschoolErythrocyte CountFetal HemoglobinHumansHydroxyureaalpha-Thalassemia

Pub Type(s)

Journal Article
Research Support, N.I.H., Extramural

Language

eng

PubMed ID

28783617

Citation

Figueiredo, Lisa, et al. "An Age Dependent Response to Hydroxyurea in Pediatric Sickle Cell Anemia Patients With Alpha Thalassemia Trait." Blood Cells, Molecules & Diseases, vol. 66, 2017, pp. 19-23.
Figueiredo L, Morrone K, Wei C, et al. An age dependent response to hydroxyurea in pediatric sickle cell anemia patients with alpha thalassemia trait. Blood Cells Mol Dis. 2017;66:19-23.
Figueiredo, L., Morrone, K., Wei, C., Ireland, K., Cohen, H. W., Driscoll, C., & Manwani, D. (2017). An age dependent response to hydroxyurea in pediatric sickle cell anemia patients with alpha thalassemia trait. Blood Cells, Molecules & Diseases, 66, 19-23. https://doi.org/10.1016/j.bcmd.2017.07.004
Figueiredo L, et al. An Age Dependent Response to Hydroxyurea in Pediatric Sickle Cell Anemia Patients With Alpha Thalassemia Trait. Blood Cells Mol Dis. 2017;66:19-23. PubMed PMID: 28783617.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - An age dependent response to hydroxyurea in pediatric sickle cell anemia patients with alpha thalassemia trait. AU - Figueiredo,Lisa, AU - Morrone,Kerry, AU - Wei,Catherine, AU - Ireland,Karen, AU - Cohen,Hillel W, AU - Driscoll,Catherine, AU - Manwani,Deepa, Y1 - 2017/07/31/ PY - 2017/04/21/received PY - 2017/07/29/revised PY - 2017/07/29/accepted PY - 2017/8/8/pubmed PY - 2018/8/23/medline PY - 2017/8/8/entrez KW - Alpha thalassemia KW - Hydroxyurea KW - Sickle cell anemia SP - 19 EP - 23 JF - Blood cells, molecules & diseases JO - Blood Cells Mol Dis VL - 66 N2 - Hydroxyurea (HU) is a key drug therapy for individuals with sickle cell anemia (SCA), yet its clinical and hematologic responses can be variable. Various studies have reported the role of α-thalassemia as one of the most prevalent heritable traits that may modify HU response. We provide data from 62 pediatric and adolescent patients with SCA, 26 with co-inherited α-thalassemia trait. Our data suggest that altered hematologic and clinical responses to HU therapy are noted in adolescent SCA individuals with co-inherited α-thalassemia trait. Adolescent patients who co-inherited α-thalassemia trait had a greater reduction in vaso-occlusive episodes compared to those without α-thalassemia, despite a less robust fetal hemoglobin induction as well as a lower maximum HU dose. This clinical improvement was associated with a lower MCH and higher RBC count. Responses to HU in younger SCA children (ages 5-11years) with co-inherited α-thalassemia trait, compared to those without α-thalassemia trait, did not show any difference in number vaso-occlusive episodes, fetal hemoglobin induction and change in MCH and RBC count. SN - 1096-0961 UR - https://www.unboundmedicine.com/medline/citation/28783617/An_age_dependent_response_to_hydroxyurea_in_pediatric_sickle_cell_anemia_patients_with_alpha_thalassemia_trait_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1079-9796(17)30185-7 DB - PRIME DP - Unbound Medicine ER -