Anemia de células falciformes con persistencia de hemoglobina fetal como factor protector: reporte de caso.

Sickle cell anemia is an autosomal recessive genetic disease characterized by the presence of hemoglobin sickle in erythrocytes. A mutation in the sixth codon of the gene for β-globin causes an abnormal hemoglobin synthesis or hemoglobin sickle. In children, the acute complications are severe pain crisis, severe infections, episodes of acute anemia and severe occlusive vessel accidents especially neurological. This rare condition of hereditary persistence of fetal hemoglobin is uncommon in people with sickle cell anemia. Clinical case: We present a case of sickle cell anemia with persistence of fetal hemoglobin in pediatric patient with a history of severe anemic syndrome, lipomeningocele and bilateral clubfoot. The short evolution of sickle cell anemia, presenting moderate splenomegaly without painful vaso-occlusive crises, infections, without any acute or chronic complications of sickle cell anemia. Conclusion: The fetal hemoglobin has a protective factor in the neonatal period and in the first months of life in children with sickle cell anemia in which, due to the level of fetal hemoglobin is slightly superior to the hemoglobin sickle, they do not have sickle cell anemia crisis until the levels of hemoglobin sickle are superior to the fetal hemoglobin. The above, led to suspect that the persistence of fetal hemoglobin inhibits the polymerization of hemoglobin sickle, which explains its potential beneficial effect. [ABSTRACT FROM AUTHOR]