Abstract | BACKGROUND: 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 beta-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 beta-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 micrograms per L (p < 0.0001), with one-half of patients achieving serum ferritin levels lower than 1000 micrograms 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.
|
Authors | M Cazzola, C Borgna-Pignatti, F Locatelli, L Ponchio, Y Beguin, P De Stefano |
Journal | Transfusion
(Transfusion)
Vol. 37
Issue 2
Pg. 135-40
(Feb 1997)
ISSN: 0041-1132 [Print] United States |
PMID | 9051086
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
|
Chemical References |
- Hemoglobins
- Receptors, Transferrin
- Siderophores
- Deferoxamine
|
Topics |
- Adolescent
- Blood Transfusion
- Child
- Deferoxamine
(therapeutic use)
- Erythropoiesis
(physiology)
- Growth
- Hemoglobins
(analysis)
- Humans
- Iron Overload
(therapy)
- Receptors, Transferrin
(blood)
- Siderophores
(therapeutic use)
- Transfusion Reaction
- Ventricular Dysfunction
(etiology)
- beta-Thalassemia
(blood, metabolism, therapy)
|