[Status of iron metabolism and erythropoietic proliferation in children with various genotypes of thalassemia].
OBJECTIVE
To study the status of iron metabolism and erythropoietic proliferation in children with various genotypes of thalassemia.


METHODS
Serum concentrations of ferritin (SF), transferrin receptor (sTfR) and erythropoietin (EPO) were measured in 158 children with thalassemia. The differences in the concentrations of the three indices among children with different genotypes of thalassemia were compared. The correlations of the hemoglobin level with sereum SF, sTfR and EPO levels were assessed.


RESULTS
Among the 158 children with thalassemia, 52(32.9%) were diagnosed with alpha-thalassemia minor, 27(17.1%) with HbH disease, 59(37.4%) with beta-thalassemia minor, 13(8.2%) with beta-thalassemia major, and 7(4.4%) with combining alpha beta thalassemia. The SF levels in children with HbH disease or beta-thalassemia major were significantly higher than those in the other thalassemia groups (P<0.01). The sTfR levels in children with beta-thalassemia major were the highest when compared with those in the other thalassemia groups (P<0.05). The EPO levels in children with beta-thalassemia major were also the highest when compared with those in the other thalassemia groups (P<0.01). There was a negative correlation between hemoglobin and EPO levels in children with HbH disease (r=-0.656, P<0.01) and beta-thalassemia major (r=-0.641; P<0.05).


CONCLUSIONS
The status of iron metabolism and erythropoietic proliferation is different in children with different genotypes of thalassemia. A combined measurement of SF, sTfR and EPO may reflect the status of erythropoietic proliferation.
Mai
Hui-Rong
HR
Department of Hematology, Shenzhen Children's Hospital, Shenzhen, Guangdong 518026, China.
Li
Chang-Gang
CG
Wang
Ying
Y
Shi
Hong-Song
HS
Zhao
Wei-Ling
WL
Chen
Yun-Sheng
YS
Chen
Xiao-Wen
XW
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D004920:Erythropoiesis; D004921:Erythropoietin / Q000097:blood; D005260:Female; D005293:Ferritins / Q000097:blood*; D005838:Genotype; D006801:Humans; D007223:Infant; D007501:Iron / Q000378:metabolism*; D008297:Male; D011990:Receptors, Transferrin / Q000097:blood; D013789:Thalassemia / Q000097:blood* / Q000378:metabolism
D011990:Receptors, Transferrin; D004921:Erythropoietin; D005293:Ferritins; D007501:Iron