Abstract

GARCIA PERALTA, Tania et al. Contributions to the study of drepanocytosis: Clinical and hematological analysis in the first 5 years of life. Rev Cubana Hematol Inmunol Hemoter [online]. 1999, vol.15, n.2, pp. 96-104. ISSN 1561-2996.

104 patients under 6 were studied. In 93,1 % of them the diagnosis was made before the first year of life, whereas in 47.6 % it was prenatal. The median of follow-up was 31 months (1-108). The highest incidence of clinical events in sickle cell anemia (SCA) occurred between 1 and 3 years old. 91.6 % of the infections were pulmonary and 21.7 % of the respiratory episodes were considered as acute chest syndrome. The events in hemoglobinopathy SC (HSC) were less frequent than in sickle cell anemia. Only one patient died in a period of almost 10 years since the study began. Hemoglobin and fetal hemoglobin (HbF) were higher among females with SCA. The commonest haplotypes of the block of genes b were Benin and Bantu. It was observed a frequency of thalassemia of 23.8 % in sickle cell anemia and of 23.7 % in HSC. Bantu and non-Bantu haplotypes and a thalassemia were not associated with the clinical manifestations or with the hematological parameters. The systematic follow-up of the patients since the first years of life reduces mortality in this period

Keywords : ANEMIA, SICKLE CELL [blood]; HEMOGLOBIN C [blood]; BETA-THALASSEMIA [blood].

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