Serum erythropoietin and erythropoiesis in high- and low-fetal hemoglobin β-thalassemia intermedia patients

Renzo Galanello, Susanna Barella, Maria P. Turco, Nicolina Giagu, Antonio Cao, Fausto Dore, Nicola L. Liberato, Roberta Guarnone, Giovanni Barosi

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Abstract

Clinical data suggest that in β-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 β- thalasemia-intermedia patients. Twenty-four showed more than 40% HbF (21 of whom with β*-thalassemia) and 6 presented lower HbF levels (β+- 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%.

Original languageEnglish
Pages (from-to)561-565
Number of pages5
JournalBlood
Volume83
Issue number2
Publication statusPublished - Jan 15 1994

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Fetal Hemoglobin
Erythropoiesis
beta-Thalassemia
Erythropoietin
Serum
Anemia
Thalassemia
Transferrin Receptors
Regression analysis
Iron-Deficiency Anemias
Iron
Reference Values
Multivariate Analysis
Regression Analysis

ASJC Scopus subject areas

  • Hematology

Cite this

Galanello, R., Barella, S., Turco, M. P., Giagu, N., Cao, A., Dore, F., ... Barosi, G. (1994). Serum erythropoietin and erythropoiesis in high- and low-fetal hemoglobin β-thalassemia intermedia patients. Blood, 83(2), 561-565.

Serum erythropoietin and erythropoiesis in high- and low-fetal hemoglobin β-thalassemia intermedia patients. / Galanello, Renzo; Barella, Susanna; Turco, Maria P.; Giagu, Nicolina; Cao, Antonio; Dore, Fausto; Liberato, Nicola L.; Guarnone, Roberta; Barosi, Giovanni.

In: Blood, Vol. 83, No. 2, 15.01.1994, p. 561-565.

Research output: Contribution to journalArticle

Galanello, R, Barella, S, Turco, MP, Giagu, N, Cao, A, Dore, F, Liberato, NL, Guarnone, R & Barosi, G 1994, 'Serum erythropoietin and erythropoiesis in high- and low-fetal hemoglobin β-thalassemia intermedia patients', Blood, vol. 83, no. 2, pp. 561-565.
Galanello R, Barella S, Turco MP, Giagu N, Cao A, Dore F et al. Serum erythropoietin and erythropoiesis in high- and low-fetal hemoglobin β-thalassemia intermedia patients. Blood. 1994 Jan 15;83(2):561-565.
Galanello, Renzo ; Barella, Susanna ; Turco, Maria P. ; Giagu, Nicolina ; Cao, Antonio ; Dore, Fausto ; Liberato, Nicola L. ; Guarnone, Roberta ; Barosi, Giovanni. / Serum erythropoietin and erythropoiesis in high- and low-fetal hemoglobin β-thalassemia intermedia patients. In: Blood. 1994 ; Vol. 83, No. 2. pp. 561-565.
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abstract = "Clinical data suggest that in β-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 β- thalasemia-intermedia patients. Twenty-four showed more than 40{\%} HbF (21 of whom with β*-thalassemia) and 6 presented lower HbF levels (β+- 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{\%}.",
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