Anemia hemolítica grave causada por hemoglobina Hammersmith. Severe hemolytic anemia due to hemoglobin Hammersmith

EBERLE EANDI, SILVIA J.; NOGUERA, NÉLIDA I.; CALVO, KARINA L.; OJEDA, MARÍA; BRAGÓS, IRMA; PRATTI, ARIANNA; MILANI, ANGELA; TARGOVNIK , HÉCTOR M.; SCIUCCATI, GABRIELA; DÍAZ, LILIAN; BONDUEL, MARIANA; FELIU-TORRES, AURORA

ARCHIVOS ARGENTINOS DE PEDIATRIA

SOC ARGENTINA PEDIATRIA

Lugar: Buenos Aires, Argentina; Año: 2009 vol. 107 p. 347 - 352

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 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 150 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 Hammersmith) in a girl with severe hemolytic anemia, splenomegaly and red blood cell requirement