Emerging insights in the management of hemoglobin E beta thalassemia.
Globally, hemoglobin (Hb) E beta thalassemia accounts for approximately half the severe forms of beta thalassemia. Because of its wide clinical diversity and the ability of patients with this condition to adapt unusually well to low hemoglobin levels, the management of Hb E beta thalassemia, particularly the decision to instigate regular blood transfusion, is particularly difficult. Here, we present a summary of our work in patients with this condition, which attempts to define clinical, adaptive, and genetic factors of possible value in determining the early management of this condition.
Olivieri
Nancy F
NF
Hemoglobinopathy Research Program, Toronto General Hospital, University of Toronto, Toronto, Ontario, Canada. noliv@attglobal.net
Thayalsuthan
Vivekanandan
V
O'Donnell
Angela
A
Premawardhena
Anuja
A
Rigobon
Christopher
C
Muraca
Giulia
G
Fisher
Christopher
C
Weatherall
David J
DJ
D001803:Blood Transfusion; D006446:Hemoglobin E; D006801:Humans; D017086:beta-Thalassemia / Q000503:physiopathology / Q000628:therapy*
D006446:Hemoglobin E