Ruiz CED, Hernández MA, Nieva GB, García AJ, Hernández CC, Salamanca GF, Peñaloza ERI

Language: Spanish
References: 25
Page: 299-304
PDF size: 65.01 Kb.

ABSTRACT

Introduction: Sickle cell anemia is caused by a variant of the Β-globin gene called sickle hemoglobin (HbS) that in homozygote state is translated into polymerization of hemoglobin molecules, deforming erythrocytes and producing episodes of vaso-occlusion, painful crises, and frequent infections, with high morbidity and mortality. Some factors modify disease evolution, producing less severe clinical course, for example, high levels of fetal hemoglobin (HbF) and Senegal and Arabia/India haplotypes. Objective: To compare clinical severity of the disease in patients with sickle cell anemia and high levels of HbF versus sickle cell anemia and low levels of HbF. Material and methods: A total of 24 patients homozygous for HbS were confirmed by electrophoresis in cellulose acetate to pH acid and alkaline. Clinical and hematologic characteristics of each patient were analyzed, forming two groups: 1) Patients with sickle cell disease and high HbF. 2) Patients with sickle cell disease and low HbF. Results: Patients with sickle cell anemia and high levels of HbF presented less clinical severity when compared with those who presented low HbF. Clinical and hematologic characteristics with differences significant between both groups were painful crises, infections, hemoglobin leve Conclusions: Patients with sickle cell anemia and high levels of fetal hemoglobin have less clinical severity than those with low levels of fetal hemoglobin.

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