ABSTRACT
CONTEXT AND OBJECTIVES Sickle cell disease (SCD) is the most common genetic disorder among people of African descent, affecting approximately 3,500 newborns each year in Brazil. Hydroxyurea (HU) is the only effective drug to treating patients with SCD, thereby reducing morbidity and mortality. The objective was to analyze the effects of HU on SCD patients at our institution. DESIGN AND SETTING Retrospective study conducted at a sickle cell centre in Ribeirão Preto, São Paulo, Brazil. METHODS We analyzed clinical and laboratory data on 37 patients. The hematological parameters and clinical events that occurred during the year before and the first year of treatment with HU were analyzed. The mean dose of HU was 24.5 ± 5.5 mg/kg/day. RESULTS There were rises in three parameters: hemoglobin (8.3 g/dl to 9.0 g/dl, P = 0.0003), fetal hemoglobin (HbF) (2.6% to 19.8%, P < 0.0001) and mean cell volume MCV (89 to 105 fl, P = 0.001); and reductions in the numbers of leukocytes (10,050/µl to 5,700/µl, P < 0.0001), neutrophils (6,200/µl to 3,400/µl, P = 0.001), platelets (459,000/µl to 373,000/µl, P = 0.0002), painful crises (1.86 to 0.81, P = 0.0014), acute chest syndromes (0.35 to 0.08, P = 0.0045), infections (1.03 to 0.5, P = 0.047), hospitalizations (1.63 to 0.53, P = 0.0013) and transfusions (1.23 to 0.1, P = 0.0051). CONCLUSION The patients presented clinical and hematological improvements, with an increase in HbF and a reduction in the infection rate, which had not been addressed in most previous studies. .
CONTEXTO E OBJETIVO A doença falciforme (SCD) é o distúrbio genético mais comum entre afrodes-cendentes, afetando aproximadamente 3.500 recém-nascidos a cada ano no Brasil. A hidroxiureia (HU) é a única droga efetiva para o tratamento dos pacientes com SCD, reduzindo a morbidade e a mortalidade da doença. O objetivo do estudo foi analisar os efeitos da HU em pacientes com SCD em nossa instituição. TIPO DE ESTUDO E LOCAL Estudo retrospectivo realizado em um centro de anemia falciforme em Ribeirão Preto, São Paulo, Brasil. MÉTODOS Nós analisamos os dados clínicos e laboratoriais de 37 pacientes. Os parâmetros hematológicos e eventos clínicos que ocorreram no ano anterior e durante o primeiro ano de tratamento com HU foram analisados. A dose média de HU foi 24.5 ± 5.5 mg/kg/dia. RESULTADOS Houve aumento em três parâmetros estudados: hemoglobina (8,3 g/dl para 9,0 g/dl, P = 0,0003), hemoglobina fetal (HbF) (2,6% para 19,8%, P < 0,0001) e volume corpuscular médio (VCM) (89 para 105 fl, P = 0,001); e redução do número de leucócitos (10.050/µl para 5.700/µl, P < 0,0001), neutrófilos (6.200/µl para 3.400/µl, P = 0,001), plaquetas (459.000/µl para 373.000/µl, P = 0,0002), crises dolorosas (1,86 para 0,81, P = 0,0014), síndrome torácica aguda (0,35 para 0,08, P = 0,0045), infecções (1,03 para 0,5, P = 0,047), hospitalizações (1,63 para 0,53, P = 0,0013) e número de transfusões (1,23 para 0,1, P = 0,0051). CONCLUSÃO Os pacientes apresentaram melhora clínica e hematológica, com aumento da HbF e redução da taxa de infecção, dado este não explorado na maioria dos estudos clínicos já publicados. .
Subject(s)
Adolescent , Adult , Child , Female , Humans , Male , Young Adult , Anemia, Sickle Cell/drug therapy , Antisickling Agents/therapeutic use , Hydroxyurea/therapeutic use , Analysis of Variance , Anemia, Sickle Cell/blood , Antisickling Agents/pharmacology , Blood Transfusion , Brazil , Erythrocyte Indices/drug effects , Fetal Hemoglobin/drug effects , Hemoglobin, Sickle/drug effects , Hydroxyurea/pharmacology , Retrospective Studies , Statistics, Nonparametric , Time Factors , Treatment OutcomeABSTRACT
Production of fetal proteins by malignant tissue is a recognized phenomenon seen in many neoplastic disorders. Hence, the aim was to determine if there is a positive correlation between administration of chemotherapy and reduction in fetal hemoglobin [HbF] levels. In a prospective study at the University of Benin Teaching Hospital, Nigeria, 50 cancer patients at various stages of the disease and 50 controls for 10 months period [March to December, 2005], HbF was determined in pre- chemotherapy [n=23] and post-chemotherapy [n=27] cancer patients. Fetal hemoglobin was estimated by the modified Betke's method. A total of 20 patients [40%] comprising 10 pre-chemotherapy and 10 post-chemotherapy patients presented with increased HbF which was statistically significant [p<0.0001]. There was also a significant decrease in post- chemotherapy result when both pre and post-chemotherapy values were compared [p=0.0073]. The results presented indicate that during recovery of erythropoiesis some erythroblasts synthesized HbF and there was a reduction following administration of chemotherapy