Publication: Benefits of chronic blood transfusion in hemoglobin E/β thalassemia with pulmonary arterial hypertension
Issued Date
2014-08-19
Resource Type
ISSN
11787074
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2-s2.0-84907335967
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Mahidol University
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SCOPUS
Bibliographic Citation
International Journal of General Medicine. Vol.7, (2014), 411-416
Suggested Citation
Nonlawan Chueamuangphan, Jayanton Patumanond, Wattana Wongtheptien, Weerasak Nawarawong, Apichard Sukonthasarn, Suporn Chuncharunee, Chamaiporn Tawichasri Benefits of chronic blood transfusion in hemoglobin E/β thalassemia with pulmonary arterial hypertension. International Journal of General Medicine. Vol.7, (2014), 411-416. doi:10.2147/IJGM.S66610 Retrieved from: https://repository.li.mahidol.ac.th/handle/20.500.14594/34213
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Title
Benefits of chronic blood transfusion in hemoglobin E/β thalassemia with pulmonary arterial hypertension
Abstract
Objective: The aim of the research reported here was to compare pulmonary artery systolic pressure (PASP) and 6-minute walk distance after 1 year of follow-up in hemoglobin E/β thalassemia (E/β-Thal) with pulmonary arterial hypertension (PAH) patients who received chronic blood transfusions versus those who received occasional transfusions. Methods: A nonrandomized clinical trial was conducted at the Hematological Outpatient Clinic of Chiang Rai Hospital, Thailand. All adult cases of E/β-Thal with PAH (defined as PASP >35 mmHg by Doppler echocardiography) were evaluated and followed for the next 12 months. The patients were classified into two groups by patient preference. Group 1 patients received chronic blood transfusions - one to two units of leukocyte-poor packed red cells every 2-4 weeks - over 1 year to maintain pre-transfusion hemoglobin levels of ≥7.0 g/dL. Group 2 patients received occasional transfusions over the course of 1 year, with more than 4 weeks between transfusions. All patients were treated with iron chelation when serum ferritin levels were > 1,000 μg/dL. PASP and the 6-minute walk distance were evaluated at baseline and at 6 and 12 months. Propensity score adjustment was used to control for confounding by indication and contraindication. Multivariable regression analysis was used to evaluate the effects of chronic blood transfusion. Results: There were 16 (53.3%) patients in Group 1 and 14 (46.7%) in Group 2. At 12 months, patients in Group 1 had a greater reduction in PASP than those in Group 1 (adjusted mean difference, -16.83; 95% confidence interval, -26.35 to -7.32; P=0.001). The 6-minute walk distance at 12 months in Group 1 patients was greater than that in Group 2 patients (adjusted mean difference, 46.55; 95% confidence interval, 18.08 to 75.02; P=0.001). Conclusion: This study found evidence that chronic blood transfusions may have beneficial effects in PAH in thalassemia patients over 1 year. © 2014 Chueamuangphan et al.