<abstract xmlns="http://www.w3.org/1999/xhtml">Transfusion of patients with sickle cell disease (SCD) has been a challenge in clinical transfusion medicine, especially when the required donor RBCs must be U– and negative for high-prevalence Rh phenotypes (hrB, hrS). It is now possible to genotype donors to identify or confirm Uvar and U– phenotypes, as well as Rh hrB– and hrS– phenotypes, and to characterize the different <italic>RH</italic> backgrounds found in these donors. In a preliminary study of donors registered in the American Rare Donor Program, twelve different <italic>RH</italic> backgrounds were identified in eighteen hrB– or hrS– donors. These results, summarized in the current report, confirm the heterogeneous nature of these phenotypes and are relevant for selection of donor units for patients with antibodies to high-prevalence Rh antigens. Not all phenotypically similar units will be compatible, and matching the Rh genotype of the donor to the patient is important to prevent further Rh sensitization. Most donors referred were hrB– and carry at least one hybrid <italic>RHD-CE(3-7)-D</italic> gene that encodes a variant C antigen linked to <italic>RHCE*ceS</italic> that encodes the VS+V– phenotype. Surprisingly, the majority of donors were heterozygous, some even carrying conventional alleles, suggesting that the loss of expression of the hrB epitopes on RBCs is a dominant phenotype. Although antigen-matching of patients with SCD with donors for C, E, and K antigens has decreased the incidence of alloimmunization, some patients still become immunized to Rh antigens, indicating the units were not truly matched. RH genotyping can identify those patients with SCD who carry RH alleles that encode altered C, e, or D who are at risk for production of “apparent auto” and alloantibodies to Rh antigens. RH genotyping of alloimmunized patients with SCD, partnered with genotyping of donors, can identify compatible units that would also eliminate the risk of further Rh alloimmunization. <italic><bold>Immunohematology</bold></italic><bold>2006;22:143–147.</bold></abstract>

Transfusion of patients with sickle cell disease (SCD) has been a challenge in clinical transfusion medicine, especially when the required donor RBCs must be U– and negative for high-prevalence Rh phenotypes (hrB, hrS). It is now possible to genotype donors to identify or confirm Uvar and U– phenotypes, as well as Rh hrB– and hrS– phenotypes, and to characterize the different RH backgrounds found in these donors. In a preliminary study of donors registered in the American Rare Donor Program, twelve different RH backgrounds were identified in eighteen hrB– or hrS– donors. These results, summarized in the current report, confirm the heterogeneous nature of these phenotypes and are relevant for selection of donor units for patients with antibodies to high-prevalence Rh antigens. Not all phenotypically similar units will be compatible, and matching the Rh genotype of the donor to the patient is important to prevent further Rh sensitization. Most donors referred were hrB– and carry at least one hybrid RHD-CE(3-7)-D gene that encodes a variant C antigen linked to RHCE*ceS that encodes the VS+V– phenotype. Surprisingly, the majority of donors were heterozygous, some even carrying conventional alleles, suggesting that the loss of expression of the hrB epitopes on RBCs is a dominant phenotype. Although antigen-matching of patients with SCD with donors for C, E, and K antigens has decreased the incidence of alloimmunization, some patients still become immunized to Rh antigens, indicating the units were not truly matched. RH genotyping can identify those patients with SCD who carry RH alleles that encode altered C, e, or D who are at risk for production of “apparent auto” and alloantibodies to Rh antigens. RH genotyping of alloimmunized patients with SCD, partnered with genotyping of donors, can identify compatible units that would also eliminate the risk of further Rh alloimmunization. Immunohematology2006;22:143–147.

Molecular characterization of <italic>GYPB</italic> and <italic>RH</italic> in donors in the American Rare Donor Program

Immunohematology

Molecular characterization of  and  in donors in the American Rare Donor Program

Transfusion of patients with sickle cell disease (SCD) has been a challenge in clinical transfusion medicine, especially when the required donor RBCs must be...

Molecular characterization of GYPB and RH in donors in the American Rare Donor Program

Published Online: Apr 15, 2020

Page range: 143 - 147

DOI: https://doi.org/10.21307/immunohematology-2019-372

© 2006 S. Vege, et al., published by Sciendo