title: [Severe hemolytic anemia due to hemoglobin Hammersmith].
abstract: 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.
authors.affiliation: Servicio de Hematología-Oncología, Hospital de Pediatría Prof. Dr. Juan P. Garrahan, Buenos Aires, Argentina.
authors.forename: Silvia J Eandi
authors.initials: SJ
authors.lastname: Eberle
authors.forename: Nélida
authors.initials: N
authors.lastname: Noguera
authors.forename: Karina
authors.initials: K
authors.lastname: Calvo
authors.forename: María
authors.initials: M
authors.lastname: Ojeda
authors.forename: Irma
authors.initials: I
authors.lastname: Bragós
authors.forename: Arianna
authors.initials: A
authors.lastname: Pratti
authors.forename: Angela
authors.initials: A
authors.lastname: Milani
authors.forename: Héctor
authors.initials: H
authors.lastname: Targovnik
authors.forename: Gabriela
authors.initials: G
authors.lastname: Sciuccati
authors.forename: Lilian
authors.initials: L
authors.lastname: Díaz
authors.forename: Mariana
authors.initials: M
authors.lastname: Bonduel
authors.forename: Aurora Feliú
authors.initials: AF
authors.lastname: Torres
mesh_terms: D000743:Anemia, Hemolytic / Q000097:blood* / Q000175:diagnosis / Q000601:surgery; D002675:Child, Preschool; D005260:Female; D006455:Hemoglobins, Abnormal; D006801:Humans; D012720:Severity of Illness Index
chemical_list: D006455:Hemoglobins, Abnormal; C011478:hemoglobin Hammersmith