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Title: | Blood transfusion therapy for β-thalassemia major and hemoglobin E β-thalassemia: adequacy, trends, and determinants in Sri Lanka. |
Authors: | Mettananda, S. Pathiraja, H. Peiris, R. Wickramarathne, N. Bandara, D. de Silva, U. Mettananda, C. Premawardhena, A. |
Keywords: | Hemoglobinopathies |
Issue Date: | 2019 |
Publisher: | John Wiley |
Citation: | Pediatric Blood and Cancer.2019: e27643 |
Abstract: | BACKGROUND: Regular blood transfusion therapy still remains the cornerstone in the management of β-thalassemia. Although recommendations are clear for patients with β-thalassemia major, uniform transfusion guidelines are lacking for patients with hemoglobin E β-thalassemia. In this study, we aim to describe the adequacy, trends, and determinants of blood transfusion therapy in a large cohort of pediatric patients with β-thalassemia major and hemoglobin E β-thalassemia. METHODS/PROCEDURE: This cross-sectional study was performed among all regularly transfused patents with β-thalassemia aged 2 to 18 years attending three large thalassemia centers in Sri Lanka. Data were collected using an interviewer-administered questionnaire, perusal of clinical records, and physical examination of patients by trained doctors. RESULTS: A total of 328 patients (male 47%) were recruited; 83% had β-thalassemia major, whereas 16% had hemoglobin E β-thalassemia. Sixty-one percent of patients had low pretransfusion hemoglobin levels (< 9.0 g/dL) despite receiving high transfusion volumes (> 200 mL/kg/year) by a majority (56%). Median pretransfusion hemoglobin was significantly lower in patients with hemoglobin E β-thalassemia compared with β-thalassemia major (P < 0.001); however, there was no difference in requirement for high transfusion volumes over 200 mL/kg/year in both groups (P = 0.14). Hepatomegaly and splenomegaly were more common in hemoglobin E β-thalassemia and were associated with lower pretransfusion hemoglobin. Transfusion requirements were higher among patients with hepatitis C and in those who are underweight. CONCLUSIONS: Over 60% of regularly transfused patients with β-thalassemia have low pretransfusion hemoglobin levels despite receiving large transfusion volumes. Patients with hemoglobin E β-thalassemia are undertransfused and specific recommendations should be developed to guide transfusions in these patients. |
Description: | Indexed in Medline |
URI: | http://repository.kln.ac.lk/handle/123456789/19972 |
ISSN: | 1545-5009 (Print) 1545-5017 (Electronic) |
Appears in Collections: | Journal/Magazine Articles |
Files in This Item:
File | Description | Size | Format | |
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Mettananda_et_al-2019-Pediatric_Blood_&_Cancer.pdf | 417.91 kB | Adobe PDF | View/Open |
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