Abstract

EANDI EBERLE, Silvia J. et al. Severe hemotytic anemia due to hemoglobin Hammersmith. Arch. argent. pediatr. [online]. 2009, vol.107, n.4, pp.347-349. ISSN 0325-0075.

Most of the hemoglobin variants are the result of single amino acid replacement in one of the globin chains. In many cases, these hemoglobinopathies are harmless, while in others they determine alterations in the physical and chemical properties, raising clínical manifestations of variable severity. In the unstable hemoglobinopathies, the changes reduce solubility, inducing the formation of precipítales of denaturated hemoglobin (Heinz bodies), which damage the membrane and finally destroy the red blood cells prematurely. Up to now, more than 150 different unstable hemoglobins have been described; most of them cause chronic hemolysis, increa-sed by infections or drugs. We report the clinical presentation of an unstable hemoglobin (hemoglobin Hammersmith) in a girl with severe hemolytic anemia, splenomegaly and red blood cell requirement.

Keywords : Hemolytic anemia; Unstable hemoglobin; Abnormal hemoglobin.

        · abstract in Spanish     · text in Spanish     · Spanish ( pdf )