Premium
The haemoglobinopathy survey: The reality of transfusion practice in sickle cell disease and thalassaemia in England
Author(s) -
Trompeter Sara,
Estcourt Lise,
Mora Ana,
Wong Esther,
Collett David,
BoltonMaggs Paula,
Poles Debbi,
Deary Alison,
Watt Alison
Publication year - 2020
Publication title -
transfusion medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.471
H-Index - 59
eISSN - 1365-3148
pISSN - 0958-7578
DOI - 10.1111/tme.12732
Subject(s) - medicine , exchange transfusion , blood transfusion , population , thalassemia , pediatrics , modalities , disease , hemoglobinopathy , social science , environmental health , sociology
Summary Objectives To establish, in an unselected population of London haemoglobinopathy patients, transfusion requirements, blood antigens/alloantibodies, transfusion modalities, burden of transfusion reactions and donor exposure. Background Haemoglobinopathy patients are among the most highly transfused patient populations, and the overall population and number of patients on long‐term transfusion programmes are increasing. To provide a safe and efficacious transfusion service for patients, it is important to understand current practice, morbidity associated with transfusion, efficacy of different transfusion modalities and geno‐/phenotype requirements. Methods Data on 4451 transfusion episodes in 760 patients from 12 London hospitals were collected retrospectively over a 6‐month period in 2011. Results Alloimmunisation prevalence was 17% for sickle cell disease (SCD) and 22% for thalassaemia, most commonly anti‐Rh/Kell/Kp a /C w . Rh phenotypes differed between SCD (R o r 59.8%/R 1 r 15.9%/R 2 r 15.6%) and thalassaemia (R 1 R 1 29.6%/R 1 r 28.4%/R 1 R 2 15.4%). Recording of pheno‐/genotypes fell below recommendations. A 2‐weekly manual exchange and 3‐weekly automated exchange came closest to achieving presumptive targets. In adults with thalassaemia, the mean blood requirement was 36 units per year; for SCD, erythrocytapheresis was carried out every 7 weeks with 66 units; for manual exchange, it was 38 units every 4 weeks; and for simple transfusion, it was 30 units p.a. every 4 weeks. Conclusion Transfusion modality choice was influenced by the resources available—children mostly received simple transfusions, and adults received erythrocytapheresis; the relationships between frequency of exchanges/transfusion modality/target HbA% were not simple, possibly reflecting the difference in recipient erythropoiesis and consequent transfusion modality selection bias; adherence to existing and current guidelines regarding geno‐/phenotyping was limited; and alloimmunisation had a low incidence and high prevalence in both disorders.