Abstract

ARENCIBIA-NUNEZ, Alberto et al. Hemolytic index: an approach to clinical subphenotypes in children with sickle cell disease. Rev Cubana Hematol Inmunol Hemoter [online]. 2012, vol.28, n.3, pp.263-274. ISSN 0864-0289.

A prospective study was performed in order to calculate the hemolytic index and determine its association with clinical and laboratory manifestations. Forty children with sickle cell anemia and Sß º thalassemia treated at the Institute of Hematology and Immunology from 2009 to 2010 were included in this study. The haemolytic index, with mean 0 and range between -3 to 3, was calculated from the values of lactate dehydrogenase, bilirubin and reticulocytes, using the principal component analysis. We compared patients in group 1 with less than 33 hemolytic index percentile (-3 to -0.71, n = 13), patients in group 2 with hemolytic index greater than 66 percentile (0.84 to 3; n = 14). Acute chest syndrome (38.5 % vs. 78.6% p = 0.034) and cerebrovascular disease (0 % vs. 28.6 %, P = 0.037) were more frequent in patients of group 2. The prevalence of asthma (p = 0.080) and systolic blood pressure (p = 0.076) tended to be higher in group 2; 42.9 % of patients in group 2 had tricuspid regurgitation velocity ? 2.5 m / s, which did not occur in any patient in group 1 (p = 0.007). Transfusions (p = 0.021) and the use of hydroxyurea (p = 0.081) were more frequent in group 2. The correlation of the tricuspid regurgitation velocity with haemolytic index (r = 0.61, p <0.0001) was significantly higher than other markers of hemolysis included in this analysis. This haemolytic index allows differentiating patients with hemolytic phenotype who have a characteristic clinical behavior.

Keywords : sickle cell anemia; principal component analysis; hemolysis; pulmonary hypertension.

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