Relationship between transfusion regimen and suppression of erythropoiesis in beta-thalassaemia major.
In the management of beta-thalassaemia major, different transfusion schemes are employed with baseline haemoglobin levels ranging from 8 to over 12 g/dl. We studied the relationship between transfusion regimen and suppression of erythropoiesis in 52 patients with beta-thalassaemia major whose mean pretransfusion haemoglobin levels ranged from 8.6 to 10.9 g/dl. Multiple, regression analysis showed that serum transferrin receptor was the parameter more closely related to mean pretransfusion haemoglobin (r = -0.77, P < 0.001). As measured through serum transferrin receptor, erythroid activity was 1-2 times normal for pretransfusion haemoglobin levels between 10 and 11 g/dl. 1-4 times normal for levels from 9 to 10 g/dl, and 2-6 times normal for levels from 8.6 to 9 g/dl. Mean pretransfusion haemoglobin was also inversely related to serum erythropoietin (r = -0.72, P < 0.001), whereas it showed no or a weak relationship with Hb F, reticulocyte count, or circulating nucleated red cell count. This study suggests that serum transferrin receptor is a reliable indicator of suppression of erythropoiesis in beta-thalassaemia major. On the basis of our findings, pretransfusion haemoglobin values of < or = 9 g/dl should be adopted with caution, because these levels can be associated with an insufficient inhibition of erythroid marrow expansion. However, a transfusion programme, with a baseline haemoglobin of 9-10 g/dl, may provide enough suppression of erythropoiesis and allow a reduction in blood consumption as compared with the classic hyper- or supertransfusion schemes. Since fixed haemoglobin levels may not be the best target for transfusion treatment in all thalassaemic patients, assay of serum transferrin receptor may be helpful for individualizing the transfusion regimens.
Cazzola
M
M
Department of Internal Medicine and Medical Oncology, University of Pavia, Italy.
De Stefano
P
P
Ponchio
L
L
Locatelli
F
F
Beguin
Y
Y
Dessi
C
C
Barella
S
S
Cao
A
A
Galanello
R
R
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D017707:Erythrocyte Transfusion / Q000379:methods*; D004920:Erythropoiesis / Q000502:physiology*; D004921:Erythropoietin / Q000097:blood; D006454:Hemoglobins / Q000032:analysis; D006801:Humans; D011990:Receptors, Transferrin / Q000032:analysis; D012044:Regression Analysis; D017086:beta-Thalassemia / Q000097:blood / Q000503:physiopathology* / Q000628:therapy*
D006454:Hemoglobins; D011990:Receptors, Transferrin; D004921:Erythropoietin