[Severe hemolytic anemia due to hemoglobin Southampton: case report].

Abstract

Variant hemoglobins are the result of different types of mutations that occur in the globin genes. In many cases, these hemoglobinopathies are harmless, while in others they determine alterations in the physical and chemical properties raising clinical manifestations of variable severity. In the unstable hemoglobinopathies, the changes reduce solubility, inducing the formation of precipitates of denaturated hemoglobin (Heinz bodies), which damage the membrane and finally destroy the red blood cells prematurely. Up to now, more than 142 different unstable hemoglobins have been described, most of them cause chronic hemolysis, increased by infections or drugs. We report the clinical presentation of an unstable hemoglobin (hemoglobin Southampton) in a girl with severe hemolytic anemia, splenomegaly and red blood cell requirement.

• Authors: Vanesa Avalos Gómez, Silvia Eandi Eberle, Carolina Pepe, Gabriela Sciuccati, Nerina García Rosolen, Carolina Cervio, Lilian Díaz, Andrea Candás, Mariana Bonduel, Guillermo Piazza, Darío Chaves, Aurora Feliú Torres
• Journal: Archivos argentinos de pediatria (Arch Argent Pediatr) Vol. 110 Issue 5 Pg. e91-4 (Oct 2012) ISSN: 1668-3501 [Electronic] Argentina
• Vernacular Title: Anemia hemolítica grave causada por hemoglobina Southampton: presentación de caso clínico.
• PMID: 23070193 (Publication Type: Case Reports, English Abstract, Journal Article)

Chemical References

• Hemoglobins, Abnormal
• hemoglobin Southampton

Topics

• Anemia, Hemolytic (blood, diagnosis, etiology)
• Child, Preschool
• Female
• Hemoglobins, Abnormal (analysis)
• Humans
• Severity of Illness Index