|
|
 Previous Article | Table of Contents | Next Article 
Serum erythropoietin and erythropoiesis in high- and low-fetal hemoglobin
beta-thalassemia intermedia patients
R Galanello, S Barella, MP Turco, N Giagu, A Cao, F Dore, NL Liberato, R Guarnone and G Barosi
Istituto di Clinica e Biologia dell'Eta'Evolutiva, Universita di Cagliari,
Italy.
Clinical data suggest that in beta-thalassemia-intermedia patients, higher
levels of circulating fetal hemoglobin (HbF) are associated with greater
disease severity at comparable degrees of anemia. We assessed the influence
of the amount of circulating HbF on serum erythropoietin (s-Epo) levels and
on serum transferrin receptor, a measure of erythropoiesis, in 30
beta-thalassemia-intermedia patients. Twenty-four showed more than 40% HbF
(21 of whom with beta (0)-thalassemia) and 6 presented lower HbF levels
(beta(+)-thalassemia). The two groups of patients did not differ in age
(15.3 v 19 years, respectively) or degree of anemia (Hb = 8.8 g/dL in both
groups). Log (s-Epo) was correlated inversely with Hb (r = -0.47; P <
.01), and directly with HbF (r = .55; P < .001). Multivariate regression
analysis showed that Hb and HbF were independently correlated with s-Epo
levels. High-HbF patients had greater s-Epo values at the same Hb level
than low-HbF patients. Considering that iron-deficiency anemia control
patients represented the predicted physiologic response of s-Epo to anemia,
the observed/predicted s-Epo ratio in low-HbF thalassemic patients was no
different from controls, but was increased in the high-HbF group. High- HbF
patients also showed an expansion of erythropoiesis as much as four to nine
times the normal value at the same Hb level as low-HbF patients. We
conclude that HbF exerts an independent regulatory effect on erythropoietin
production and erythropoiesis that is detectable only when HbF levels
exceed 40%.
Volume 83,
Issue 2,
pp. 561-565,
01/15/1994
Copyright © 1994 by The American Society of Hematology
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
A. O'Donnell, A. Premawardhena, M. Arambepola, S. J. Allen, T. E. A. Peto, C. A. Fisher, D. C. Rees, N. F. Olivieri, and D. J. Weatherall
Age-related changes in adaptation to severe anemia in childhood in developing countries
PNAS,
May 29, 2007;
104(22):
9440 - 9444.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Aessopos, M. Kati, and D. Farmakis
Heart disease in thalassemia intermedia: a review of the underlying pathophysiology
Haematologica,
May 1, 2007;
92(5):
658 - 665.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. P. PERRINE, S. A. CASTANEDA, M. S. BOOSALIS, G. L. WHITE, B. M. JONES, and R. BOHACEK
Induction of Fetal Globin in {beta}-Thalassemia: Cellular Obstacles and Molecular Progress
Ann. N.Y. Acad. Sci.,
November 1, 2005;
1054(1):
257 - 265.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. P. Perrine
Fetal Globin Induction--Can It Cure {beta} Thalassemia?
Hematology,
January 1, 2005;
2005(1):
38 - 44.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. Gabbianelli, U. Testa, A. Massa, O. Morsilli, E. Saulle, N. M. Sposi, E. Petrucci, G. Mariani, and C. Peschle
HbF reactivation in sibling BFU-E colonies: synergistic interaction of kit ligand with low-dose dexamethasone
Blood,
April 1, 2003;
101(7):
2826 - 2832.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
G. J. DOVER
Hemoglobin Switching Protocols in Thalassemia: Experience with Sodium Phenylbutyrate and Hydroxyurea
Ann. N.Y. Acad. Sci.,
June 30, 1998;
850(1):
80 - 86.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. Cazzola, F. Mercuriali, and C. Brugnara
Use of Recombinant Human Erythropoietin Outside the Setting of Uremia
Blood,
June 15, 1997;
89(12):
4248 - 4267.
[Full Text]
[PDF]
|
|
|
| |
