Impact of hydroxyurea on clinical and biological parameters of sickle cell anemia in children in Abidjan

Main Article Content

MIREILLE YAYO- AYE
Adia Eusèbe Adjambri
Boidy Kouakou
Rebecca N'guessan-Blao
Louis Missa Adjé
Taïratou Kamagaté
Vincent Yapo
Duni Sawadogo

Keywords

Sickle cell, Hydroxyurea, Côte d’Ivoire

Abstract

Background: The lives of individuals affected by sickle cell disease are marked by painful crises sometimes accompanied by complications. Curative treatments such as bone marrow transplantation or gene therapy exist, but are not currently performed in Côte d'Ivoire. Treatment with hydroxyurea remains an effective alternative. The aim of our study is to contribute to improving the management of children with sickle cell disease.


Methods: We conducted a prospective observational study from November 2017 to April 2019 at the at the Yopougon University Hospital. Children aged 5 to 15 years experiencing at least 3 vaso-occlusive crises (VOC) per year were included in the study after obtaining informed and written consent from their parents. Each patient received a daily dose of 15mg/kg of hydroxyurea.


Results: The mean age of the children was 9 years. More than 75% of patients were  homozygous SSFA2 major sickle cell individuals. After 6 months on hydroxyurea, our study observed rates of 84.4%, 100%, and 97.8%, respectively, for the absence of vaso-occlusive crises, hospitalization, and transfusion. Biologically, from M0 to M12 the mean hemoglobin level increased significantly, from 7.24 to 8.55 g/dL; white blood cell (WBC) and platelet counts decreased; Fetal hemoglobin (Hb F) increased significantly from 10.3% to 19.7%. Biochemical parameters within normal ranges, except for a moderate treatment-related increase in transaminases.


Conclusion: The induction of fetal hemoglobin (Hb F) production through hydroxyurea intake is the primary mechanism by which hydroxyurea modifies the pathogenesis of sickle cell disease

Downloads

Download data is not yet available.


Abstract 142
PDF Downloads 164
HTML Downloads 5

References

1. Elion J, Laurance S, Lapouméroulie C. Revue générale physiopathologie de la drépanocytose spécial drépanocytose. Med Trop 2010;70 (5): 454‑458.
2. Modell, Bernadette, et Matthew Darlison. Global epidemiology of haemoglobin disorders and derived service indicators. Bull World Health Organ 2008;86 (6): 480‑487. https://doi.org/10.2471/BLT.06.036673.
3. Cannas G, Poutrel S and Thomas X. Hydroxycarbamine: from an old drug used in malignant hemopathies to a current standard in sickle cell disease. Mediterr J Hematol Infect Dis 2017, 9(1): e2017015, DOI: http://dx.doi.org/10.4084/MJHID.2017.015.
4. Jayabose S, Tugal O, Sandoval C, Patel P, Puder D, Lin T, Visintainer P. Clinical and hematologic effects of hydroxyurea in children with sickle cell anemia. J Pediatr 1996;129:559–565.
DOI: 10.1016/s0022-3476(96)70121-x
5. Sangare A, Koffi KG, Sanogo I, Toure AH, Allangba O, Tolo A, Coulibaly FH, N’dhatz, Elenga JP. Essai thérapeutique de la buprenorphine (Temgesic) dans le traitement des crises douloureuses drépanocytaires. Méd Afr Noire 1998;45(2) :138-143. http://www.santetropicale.com/Resume/24512.pdf
6. Tshilolo L, Tomlinson G, Williams TN, Santos B, Olupot-Olupot P, Lane A, Aygun B, Stuber SE, Latham TS, McGann PT. Hydroxyurea for Children with Sickle Cell Anemia in SubSaharan Africa. N Engl J Med 2019;380(2):121–131. DOI:10.1056/NEJMoa1813598
7. Miller ST, Sleeper LA, Pegelow CH, Enos LE, Wang WC, Weiner SJ, Wethers DL, Smith J, Kinney TR. Prediction of adverse outcomes in children with sickle cell disease. N Engl J Med 2000; 342(2):83–89. DOI: 10.1056/NEJM200001133420203
8. Wang WC, Ware RE, Miller ST, Iyer RV, Casella JF, Minniti CP, Rana S, Thornburg CD, Rogers ZR, Kalpatthi RV, Barredo JC, R Clark Brown RC, Sarnaik SA, Howard TH, Wynn LW, Kutlar A, Armstrong FD, Files BA, Goldsmith JC, Waclawiw MA, Huang X, and Thompson BW for the BABY HUG Investigators. Hydroxycarbamide in very young children with sickle-cell anaemia: a multicentre, randomised, controlled trial (BABY HUG). Lancet 2011; 377:1663–1672. DOI: 10.1016/S0140-6736(11)60355-3
9. Creary SE, Beeman C, Stanek J, King K, McGann PT, O’Brien SH, Liem RI, Holl J, Badawy SM. Impact of hydroxyurea dose and adherence on hematologic outcomes for children with sickle cell anemia. Pediatr Blood Cancer 2022; 69(6): e29607. DOI:10.1002/pbc.29607
10. Carden MA, and Little J. Emerging disease-modifying therapies for sickle cell disease. Haematologica. 2019;104(9):1710-1719. DOI:10.3324/haematol.2018.207357
11. Patrick T McGann, and Russell E Ware. Hydroxyurea therapy for sickle cell anemia. Expert Opin Drug Saf 2015;14(11): 1749–1758. DOI:10.1517/14740338.2015.1088827
12. Lal A, Patterson L, Goldrich A, and Marsh A. Point-of-Care End-Tidal Carbon Monoxide Reflects Severity of Hemolysis in Sickle Cell Anemia. Pediatr Blood Cancer 2015 ; 62(5): 912–914. DOI:10.1002/pbc.25447