[Thalassemia intermedia. Report of two cases].
We report two cases of intermediate beta-thalassemia diagnosed at the age of 2 years and 3 1/2 years respectively. Characteristic features of this disease include delayed onset, moderate blood transfusion requirements, and frequent development of hypersplenism. Major iron overload develops even in patients who have received no transfusions. This disease is further characterized by significant genetic heterogeneity and by a reduction in the imbalance between produced chains and deficient chains.
Hôpital d'Enfants, Bab-Saadoun, Tunis, Tunisie.
F
F
Khaldi
F
F
Hassine
F
F
Guemira
B
B
Bennaceur
S
S
Fattoum
D002675:Child, Preschool; D003241:Consanguinity; D005319:Fetal Hemoglobin / Q000032:analysis; D006441:Hemoglobin A / Q000032:analysis; D006443:Hemoglobin A2 / Q000032:analysis; D006801:Humans; D008297:Male; D011379:Prognosis; D013789:Thalassemia / Q000097:blood / Q000175:diagnosis* / Q000235:genetics; D014416:Tunisia
D006441:Hemoglobin A; D006443:Hemoglobin A2; D005319:Fetal Hemoglobin
Thalassémie intermédiaire. A propos de deux cas.