Impact of temporary and involuntary suppression of hydroxyurea treatment on hematological and clinical parameters in patients with sickle cell disease
DOI:
https://doi.org/10.33448/rsd-v13i1.44727Keywords:
Hydroxyurea; Sickle cell disease; Therapeutic adherence.Abstract
Objective: Here we investigate the impact of involuntary and temporary interruption of hydroxyurea (HU) treatment in patients with Sickle Cell Disease (SCD). Methods: Clinical and hematological parameters were explored in 30 patients with SCD under treatment with HU. The study was performed in two different periods: 1 - During nine months of involuntary discontinuation (April/2015 to January/2016) and 2 – During nine months after resumption of the treatment (March/2016 to December/2016). Results: The resumption of HU treatment improved the hematological parameters, by increasing levels of hemoglobin (+0.65±0.71), hematocrit (+1.16±2.26), mean corpuscular volume (+8.33±5.89), mean corpuscular hemoglobin (+3.15±2.65). There was a reduction in red blood cell distribution width (-0.66±1.69), reticulocytes (-2.53±1.96), leukocytes (-1122±2764) and platelets numbers (-79.933±162.756). These was followed by a reduction in hospital admissions (-0.30±0.79) and in the number of pain episodes (-0.97±1.19), whereas the denial in episodes of pain crisis increased (+1.43±1.43). After resumption, in period 2, hemoglobin levels were inversely correlated with blood transfusion numbers (p=0.034) and acute thoracic syndrome (p=0.017). The hematocrit was inversely correlated with the number of consultations (p=0.030) and the number of times patients denied crisis (p=0.002). Conclusions: Our study shows that the treatment with HU improves clinical and haematological parameters in patients and highlights the negative effects of the discontinuation of the treatment in the quality of life in patients with SCD.
References
Arcanjo, A. K. A. (2018). Efeitos da suspensão temporária do tratamento com hidroxiureia em pacientes com anemia falciforme do hemocentro regional de Sobral-Ceará (Dissertação de mestrado). Universidade Federal do Ceará, Campus de Sobral.
Arduini, G. A. O., Rodrigues, L. P., & Marqui, A. B. T. (2017). Mortality by sickle cell disease in Brazil. Revista Brasileira de Hematologia e Hemoterapia, 12, 285-318. 10.1016/j.bjhh.2017.05.002
Ballas, S. K., McCarthy, W. F., Guo, N., Brugnara, C., & Kling, G. (2010). Early detection of response to hydroxyurea therapy in patients with sickle cell anemia. Hemoglobin, 34, 424–429. 10.3109/03630269.2010.484048
Bandeira, F. M. G. C. (2020). Hydroxyurea in sickle cell disease patients in Recife, Brazil. Revista Brasileira de Hematologia e Hemoterapia, 26(3), 189-194. 10.1016/j.bjhh.2020.03.014
Bernarde, S. F. P., Almeida, F. C., Andrade, G. A. A., Mesquita, B. F., Silveira, E. L., Freitas, M. P., & Klinger, P. H. D. S. (2021). Hydrea e neoplasia em pacientes falciforme. Hematology, Transfusion and Cell Therapy, 43(1), S484. 10.1016/j.htct.2021.06.416
Bernaudin, F., Verlhac, S., Arnaud, C., Kamdem, A., Hau, I., & Leveillé, E. (2016). Long-term treatment follow-up of children with sickle cell disease monitored with abnormal transcranial Doppler velocities. Blood, 127(15), 1814-1822. 10.1182/blood-2015-11-681396
Carneiro, J. S., Gonçalves, M. S., Albuquerque, S. R. L., Fraiji, N. A., & Neto, J. P. M. (2016). Beta-Globin Haplotypes and alpha-thalassemia 3.7Kb deletion in sickle cell disease patients from the occidental Brazilian Amazon. Journal of Hematology, 5(2), 123-128.
Gardner, K., Douiri, A., & Drasar, E. (2016). Survival in adults with sickle cell disease in a high-income setting. Blood, 128, 1436-1438. 10.1182/blood-2016-04-712367
Joep, W. R. S., David, J. M., Shyrin, C. A. T. D., Bart, J. B., & Karin, F. (2017). Pharmacotherapeutical strategies in the prevention of acute, vaso-occlusive pain in sickle cell disease: a systematic review. Blood Advances, 1, 1598-1616. 10.1182/bloodadvances.2017007515
Martins, M. M. F., & Teixeira, M. C. P. (2017). Analysis of hospitalization costs for sickle cell disease in the state of Bahia. Cadernos de Saúde Coletiva, 25, 24-30.
Moraes, V. M. S., Jasmelino, D. C. L., Barbosa, M. T. L., Lemos, K. M. G., Albuquerque, E. O. F., Silva, V. G., et al. (2022). Eficácia do crizanlizumabe no portador de doença falciforme: revisão de literatura. Hematology, Transfusion and Cell Therapy, 44, S59. 10.1016/j.htct.2022.06.162
Moreira, I. D. (2022). Parâmetros hematológicos avançados como medida de adesão a hidroxiureia em pacientes com anemia falciforme. https://repositorio.ufcspa.edu.br/jspui/bitstream/123456789/1877
Montalembert, M. D., Benkerrou, M., Grosse, R., & Kordes, U. (2016). Assessment of Hematological Data in a Cohort of European Children with Sickle Cell Anemia Treated with Hydroxyurea: Can European Centers Apply Today the Lessons from the Twitch Study. Blood, 128, 2494-2499. 10.1182/blood-2016-05-718754
Nevitt, S. J., Ashley, P. J., & Howard, J. (2017). Hydroxyurea (hydroxycarbamide) for sickle cell disease. Cochrane Library, 2017. 10.1002/14651858.CD002202.pub3
Pereira A. S. et al. (2018). Metodologia da pesquisa científica. UFSM
Platt, O. S., Brambilla, D. J., Rosse, W. F., Milner, P. F., Castro, O., Steinberg, M. H., & Klug, P. P. (1994). Mortality in sickle cell disease. Life expectancy and risk factors for early death. The New England journal of medicine, 330(23), 1639–1644. 10.1056/NEJM199406093302303
Rhiannon, R. P., & Hurwitz, J. L. (2018). Inflammatory molecule reduction with hydroxyurea therapy in children with sickle cell anemia. Haematologica, 103, 50–54. 10.3324/haematol.2017.184788
Santana, P. G., Araujo, A. M., Pimenta, C. T., Bezerra, M. L., Junio, S. P., & Neto, V. M. (2017). Clinical and laboratory profile of patients with sickle cell anemia. Revista Brasileira de Hematologia e Hemoterapia, 39, 40-45. 10.1016/j.bjhh.2017.01.008
Silva, W. S., Lopes, T. S. L., Reis, D. S., & Barreto, D. P. S. (2022). Aspectos sociodemográficos e clínicos de pacientes com doenças falciformes dos centros de referência em Salvador, Bahia. https://pesquisa.bvsalud.org/portal/resource/pt/pps-3894
Silva, M. P. P., Silva, K. C. P. F., & Santos, W. L. (2021). Atualizações sobre anemia falciforme – hidroxiureia. Revista JRG de Estudos Acadêmicos, 4(8), 318–326. 10.5281/zenodo.4648381
Sousa, E. Q., Sales, A. A., Santos, C. V. S., Andrade, S. M., Neto, M. P. L., & Oliveira, E. H. (2020). Use of hydroxyurea in sickle cell anemia: a literature review. Research, Society and Development, 9(2). 10.33448/rsd-v9i2.2058
Steinberg, M. H., Barton, F. B., Terrin, M. L., Charache, S., & Dover, G. J. (1997). Multicenter Study of Hydroxyurea: Fetal hemoglobin in sickle cell anemia: determinants of response to hydroxyurea. Blood, 89, 1078-1088.
Vicari, P., Barreto, A. M., & Figueiredo, M. S. (2015). Effects of hydroxyurea in a population of Brazilian patients with sickle cell anemia. American Journal of Hematology, 78, 243-244. 10.1002/ajh.24011
Ware, R. E., Davis, B. R., & Schultz, W. H. (2016). Hydroxycarbamide versus chronic transfusion for maintenance of transcranial doppler flow velocities in children with sickle cell anaemia - TCD With Transfusions Changing to Hydroxyurea (TWiTCH): a multicentre, open-label, phase 3, non-inferiority trial. The Lancet, 387, 661-670. 10.1016/S0140-6736(15)01041-8
Ware, R. E., Montalembert, M., Tshilolo, L., & Abboud, M. R. (2017). Sickle cell disease. The Lancet, 390, 311-323. 10.1016/S0140-6736(17)30193-9
World Health Organization. (2017). Model List of Essential Medicines. http://www.who.int/medicines/publications/essentialmedicines
Zambon, A. (2015). Critical shortages of new medicines in Brazil: the example of daptomycin. Revista Panamericana de Salud Pública, 38(4), 304-310. 10.17533/udea.rpsp.v38n4a03
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Copyright (c) 2024 Ana Kélvia Araújo Arcanjo ; Eric Arcanjo Bringel; José Jackson do Nascimento Costa; Alaíde Maria Rodrigues Pinheiro; Antônia Moemia Lúcia Rodrigues Portela; Júlia Oliveria de Assis; Vicente de Paulo Teixeira Pinto
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