Transfusing children with sickle cell disease using blood group genotyping when the pool of Black donors is limited

Transfusion. 2024 Apr;64(4):716-726. doi: 10.1111/trf.17778. Epub 2024 Mar 18.

Abstract

Background: Red blood cell transfusion is an effective treatment for patients with sickle cell disease (SCD). Alloimmunization can occur after a single transfusion, limiting further usage of blood transfusion. It is recommended to match for the ABO, D, C, E, and K antigens to reduce risks of alloimmunization. However, availability of compatible blood units can be challenging for blood providers with a limited number of Black donors.

Study design and methods: A prospective cohort of 205 pediatric patients with SCD was genotyped for the RH and FY genes. Transfusion and alloimmunization history were collected. Our capacity to find RhCE-matched donors was evaluated using a database of genotyped donors.

Results: Nearly 9.8% of patients carried a partial D variant and 5.9% were D-. Only 45.9% of RHCE alleles were normal, with the majority of variants affecting the RH5 (e) antigen. We found an alloimmunization prevalence of 20.7% and a Rh alloimmunization prevalence of 7.1%. Since Black donors represented only 1.40% of all blood donors in our province, D- Caucasian donors were mostly used to provide phenotype matched products. Compatible blood for patients with rare Rh variants was found only in Black donors. A donor with compatible RhCE could be identified for all patients.

Conclusion: Although Rh-compatible donors were identified, blood units might not be available when needed and/or the extended phenotype or ABO group might not match the patient. A greater effort has to be made for the recruitment of Black donors to accommodate patients with SCD.

Keywords: RH variant; alloimmunization; compatibility; genotype; sickle cell disease.

MeSH terms

  • ABO Blood-Group System / genetics
  • Anemia, Hemolytic, Autoimmune*
  • Anemia, Sickle Cell* / genetics
  • Anemia, Sickle Cell* / therapy
  • Blood Donors
  • Child
  • Genotype
  • Humans
  • Isoantibodies
  • Prospective Studies
  • Rh-Hr Blood-Group System / genetics

Substances

  • Rh-Hr Blood-Group System
  • ABO Blood-Group System
  • Isoantibodies