BACKGROUND: Hypertransfusion with a baseline hemoglobin of 10 to 12 g per dL is still considered by many to be the mainstay of conservative therapy for β-thalassemia major. However, this regimen is frequently associated with manifestations of transfusion iron overload, despite regular chelation therapy with subcutaneous desferoxamine. STUDY DESIGN AND METHODS: To verify whether a transfusion regimen with a target pretransfusion hemoglobin level between 9 and 10 g per dL can allow a significant reduction in blood consumption, while still effectively suppressing erythropoiesis, the records were reviewed of 32 β-thalassemia major patients, who were maintained at a pretransfusion hemoglobin of 11.3 ± 0.5 g per dL between 1981 and 1986. These patients were switched at the beginning of 1987 to a transfusion regimen with pretransfusion hemoglobin of 9.4 ± 0.4 g per dL. The degree of erythroid marrow activity was evaluated in these patients and in 32 subjects with β-thalassemia intermedia through the simple measurement of serum transferrin receptor. RESULTS: After the adoption of the moderate transfusion regimen, transfusion requirements decreased from 137 ± 26 to 104 ± 23 mL per kg per year of red cells (p<0.0001), and mean serum ferritin decreased from 2448 ± 1515 to 1187 ± 816 μg per L (p<0.0001), with one-half of patients achieving serum ferritin levels lower than 1000 μg per L. The proportion of patients having spontaneous pubertal development increased significantly (p<0.01), as a result of less iron-related gonadotropin insufficiency. At the lower pretransfusion hemoglobin, erythroid marrow activity did not exceed two to three times normal levels in most subjects. CONCLUSION: As compared with hypertransfusion, moderate transfusion may allow more effective prevention of iron loading, with higher likelihood of spontaneous pubertal development and without producing excessive expansion of erythropoiesis.

A moderate transfusion regimen may reduce iron loading in β-thalassemia major without producing excessive expansion of erythropoiesis / Cazzola, M.; Borgna-Pignatti, C.; Locatelli, F.; Ponchio, L.; Beguin, Y.; De Stefano, P.. - In: TRANSFUSION. - ISSN 0041-1132. - 37:2(1997), pp. 135-140. [10.1046/j.1537-2995.1997.37297203514.x]

A moderate transfusion regimen may reduce iron loading in β-thalassemia major without producing excessive expansion of erythropoiesis

Locatelli F.;
1997

Abstract

BACKGROUND: Hypertransfusion with a baseline hemoglobin of 10 to 12 g per dL is still considered by many to be the mainstay of conservative therapy for β-thalassemia major. However, this regimen is frequently associated with manifestations of transfusion iron overload, despite regular chelation therapy with subcutaneous desferoxamine. STUDY DESIGN AND METHODS: To verify whether a transfusion regimen with a target pretransfusion hemoglobin level between 9 and 10 g per dL can allow a significant reduction in blood consumption, while still effectively suppressing erythropoiesis, the records were reviewed of 32 β-thalassemia major patients, who were maintained at a pretransfusion hemoglobin of 11.3 ± 0.5 g per dL between 1981 and 1986. These patients were switched at the beginning of 1987 to a transfusion regimen with pretransfusion hemoglobin of 9.4 ± 0.4 g per dL. The degree of erythroid marrow activity was evaluated in these patients and in 32 subjects with β-thalassemia intermedia through the simple measurement of serum transferrin receptor. RESULTS: After the adoption of the moderate transfusion regimen, transfusion requirements decreased from 137 ± 26 to 104 ± 23 mL per kg per year of red cells (p<0.0001), and mean serum ferritin decreased from 2448 ± 1515 to 1187 ± 816 μg per L (p<0.0001), with one-half of patients achieving serum ferritin levels lower than 1000 μg per L. The proportion of patients having spontaneous pubertal development increased significantly (p<0.01), as a result of less iron-related gonadotropin insufficiency. At the lower pretransfusion hemoglobin, erythroid marrow activity did not exceed two to three times normal levels in most subjects. CONCLUSION: As compared with hypertransfusion, moderate transfusion may allow more effective prevention of iron loading, with higher likelihood of spontaneous pubertal development and without producing excessive expansion of erythropoiesis.
1997
...
01 Pubblicazione su rivista::01a Articolo in rivista
A moderate transfusion regimen may reduce iron loading in β-thalassemia major without producing excessive expansion of erythropoiesis / Cazzola, M.; Borgna-Pignatti, C.; Locatelli, F.; Ponchio, L.; Beguin, Y.; De Stefano, P.. - In: TRANSFUSION. - ISSN 0041-1132. - 37:2(1997), pp. 135-140. [10.1046/j.1537-2995.1997.37297203514.x]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/1488969
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