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