Blood transfusion therapy for ß-thalassemia major and hemoglobin E ß-thalassemia: Adequacy, trends, and determinants in Sri Lanka.
Pediatr Blood Cancer
; 66(5): e27643, 2019 05.
Article
en En
| MEDLINE
| ID: mdl-30697927
ABSTRACT
BACKGROUND:
Regular blood transfusion therapy still remains the cornerstone in the management of ß-thalassemia. Although recommendations are clear for patients with ß-thalassemia major, uniform transfusion guidelines are lacking for patients with hemoglobin E ß-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 ß-thalassemia major and hemoglobin E ß-thalassemia. METHODS/PROCEDURE This cross-sectional study was performed among all regularly transfused patents with ß-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 ß-thalassemia major, whereas 16% had hemoglobin E ß-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 ß-thalassemia compared with ß-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 ß-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 ß-thalassemia have low pretransfusion hemoglobin levels despite receiving large transfusion volumes. Patients with hemoglobin E ß-thalassemia are undertransfused and specific recommendations should be developed to guide transfusions in these patients.Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Transfusión Sanguínea
/
Hemoglobina E
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Talasemia beta
Tipo de estudio:
Guideline
/
Incidence_studies
/
Observational_studies
/
Prevalence_studies
/
Prognostic_studies
/
Qualitative_research
/
Risk_factors_studies
Límite:
Adolescent
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Child
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Child, preschool
/
Female
/
Humans
/
Male
País/Región como asunto:
Asia
Idioma:
En
Revista:
Pediatr Blood Cancer
Asunto de la revista:
HEMATOLOGIA
/
NEOPLASIAS
/
PEDIATRIA
Año:
2019
Tipo del documento:
Article
País de afiliación:
Sri Lanka