Palavras-chave
Hidroxiuréia
Hemoglobina fetal
Hemoglobinopatias
Como Citar
Copyright © | Todos os direitos reservados.
A revista detém os direitos autorais exclusivos de publicação deste artigo nos termos da lei 9610/98.
Reprodução parcial
É livre o uso de partes do texto, figuras e questionário do artigo, sendo obrigatória a citação dos autores e revista.
Reprodução total
É expressamente proibida, devendo ser autorizada pela revista.
Resumo
Objetivo: Caracterizar o perfil clínico e laboratorial de pacientes com Anemia Falciforme (AF) em uso de hidroxiureia (HU) tratados na Fundação Centro de Hemoterapia e Hematologia do Pará (HEMOPA). Métodos: Trata-se de um estudo analítico, retrospectivo e comparativo, baseado na análise de prontuários e dados laboratoriais de pacientes adultos com genótipo HbSS acompanhados por dois anos. A amostra foi composta por 120 indivíduos, divididos em um grupo tratado com HU (n=93) e um grupo sem tratamento (n=27). Foram avaliados parâmetros clínicos como crises vaso-oclusivas, transfusões e síndrome torácica aguda, além de variáveis hematológicas e bioquímicas. Resultados: Observou-se uma redução significativa nas crises vaso-oclusivas no grupo tratado com HU, associada a um aumento progressivo da hemoglobina fetal e uma diminuição da fração de hemoglobina S. As alterações nos índices hematimétricos, com aumento do Volume Corcuspular Médio (VCM) e redução da reticulocitose, indicaram menor hemólise e maior estabilidade eritrocitária. O tratamento com HU também esteve associado a um perfil inflamatório favorável e a níveis baixos de transaminases e ureia, sem impacto negativo na função renal. Conclusão: Conclui-se que a HU promove benefícios clínicos e laboratoriais relevantes em pacientes com AF, reforçando a importância da adesão terapêutica e do acompanhamento individualizado no manejo da doença.
Referências
AGRAWAL RK, et al. Hydroxyurea in sickle cell disease: drug review. Indian Journal of Hematology and Blood Transfusion, 2014; 30(2): 91-96.
ALKHALIFAH SA, et al. The impact of Hydroxyurea on the rates of Vaso–occlusive crises in patients with sickle cell disease in Saudi Arabia: A single–center study. BMC Emergency Medicine, 2022; 22(1): 188.
AL-SHAMI AS, et al. Impact of Hydroxyurea on Liver, Kidney, and Hematological Parameters in Sickle Cell Anemia Patients Above 15 Years Old in Sana’a City. International Journal of General Medicine, 2025: 6989-7001.
COKIC VP, et al. Hydroxyurea induces fetal hemoglobin by the nitric oxide–dependent activation of soluble guanylyl cyclase. The Journal of clinical investigation, 2003; 111(2): 231-239.
CONNES P, et al. Vascular pathophysiology of sickle cell disease. La Presse Médicale, 2023; 52(4): 104202.
DE MONTALEMBERT M, et al. Real‐life experience with hydroxyurea in patients with sickle cell disease: results from the prospective ESCORT‐HU cohort study. American journal of hematology, 2021; 96(10): 1223-1231.
DÍAZ-MATALLANA M, et al. Disease genotype, haplotypes, diagnosis and associated studies in sickle cell anemia. Revista Médica de Chile, 2021; 149(9): 1322-1329.
DOBIE G. Sickle Cell Disease and Thromboembolism: New Insights on the Pathophysiology, Diagnosis, and Treatment. Clinical Laboratory, 2023; 69(7).
DOS SANTOS SANT’ANA PG, et al. Clinical and laboratory profile of patients with sickle cell anemia. Revista brasileira de hematologia e hemoterapia, 2017; 39(1): 40-45.
ELENDU C, et al. Understanding sickle cell disease: causes, symptoms, and treatment options. Medicine, 2023;102(38): e35237.
FOGLIAZZA F, et al. Chronic Hydroxyurea Therapy in Children with Sickle Cell Anemia: Mechanisms of Action, Systemic Effects, and Long-Term Safety. Journal of Clinical Medicine, 2025; 14(23): 8599.
GANGJI A, et al. Effects of hydroxyurea treatment on haemolysis in patients with sickle cell disease at Muhimbili national hospital, Tanzania. Tanzania journal of science, 2021; 47(3): 1165.
GAZZA C, et al. Correlation between disease biomarkers and hemoglobin F levels in sickle cell patients. Blood, 2023; 142: 5312.
GONZÁLEZ-PÉREZ C, et al. Influence of hydroxyurea on the severity of acute chest syndrome in patients with sickle cell disease. Anales de Pediatría (English Edition), 2022; 97(1): 63-65.
KABUYI PL, et al. Hydroxyurea treatment for adult sickle cell anemia patients in Kinshasa. EJHaem, 2023; 4(3): 595–601.
KHARGEKAR N, et al. Role of hydroxyurea therapy in the prevention of organ damage in sickle cell disease: a systematic review and meta-analysis. Systematic Reviews, 2024; 13(1): 60.
LOBO C, et al. Optimization of hydroxyurea in sickle cell disease in Brazil. Hematology, Transfusion and Cell Therapy, 2025; 47(2): 103826.
LÓPEZ-RUBIO M, ARGÜELLO MARINA M. The current role of hydroxyurea in the treatment of sickle cell anemia. Journal of Clinical Medicine, 2024; 13(21): 6404.
MAHADEVIA H, et al. A review on disease modifying pharmacologic therapies for sickle cell disease. Annals of Hematology, 2025; 104(2): 881-893.
MANDAL AK, et al. Sickle cell hemoglobin. Vertebrate and Invertebrate Respiratory Proteins, Lipoproteins and other Body Fluid Proteins, 2020: 297-322.
NADER E, et al. Hydroxyurea therapy modulates sickle cell anemia red blood cell physiology: Impact on RBC deformability, oxidative stress, nitrite levels and nitric oxide synthase signalling pathway. Nitric Oxide, 2018; 81: 28-35.
OBEAGU EI, OBEAGU GU. Sickle cell anaemia in pregnancy: a review. International Research in Medical and Health Sciences, 2023; 6(2): 10-13.
PHAN V, et al. High hydroxyurea usage in sickle cell anemia regardless of patient demographics. Pediatric blood & cancer, 2024; 71(2): e30728.
POWER‐HAYS A, et al. Reducing transfusion utilization for children with sickle cell anemia in sub‐Saharan Africa with hydroxyurea: analysis from the phase I/II REACH trial. American journal of hematology, 2024; 99(4): 625-632.
PULE GD, et al. A systematic review of known mechanisms of hydroxyurea-induced fetal hemoglobin for treatment of sickle cell disease. Expert review of hematology, 2015; 8(5): 669-679.
SANTOS FKDS, MAIA CN. Patients with sickle cell disease taking hydroxyurea in the Hemocentro Regional de Montes Claros. Revista Brasileira de Hematologia e Hemoterapia, 2011; 33(2): 105-109.
SILVA-PINTO AC, et al. Clinical and hematological effects of hydroxyurea therapy in sickle cell patients: a single-center experience in Brazil. São Paulo Medical Journal, 2013; 131: 238-243.
TSHILOLO L, et al. Hydroxyurea for children with sickle cell anemia in sub-Saharan Africa. New England Journal of Medicine, 2019; 380(2): 121-131.
YAHOUÉDÉHOU SCMA, et al. Hydroxyurea alters hematological, biochemical and inflammatory biomarkers in Brazilian children with SCA: Investigating associations with βS haplotype and α-thalassemia. PLoS One, 2019; 14(7): e0218040.
ZAHRAN AM, et al. Effect of hydroxyurea treatment on the inflammatory markers among children with sickle cell disease. Clinical and Applied Thrombosis/Hemostasis, 2020; 26: 1076029619895111.