title: Analysis of RhCE variants among 806 individuals in France: considerations for transfusion safety, with emphasis on patients with sickle cell disease.
abstract: BACKGROUND
  DNA testing has enabled the documenting of numerous variants of RHCE alleles, especially in individuals of African origin. The risk for production of clinically significant alloantibodies to Rh antigens of patients carrying variant RHCE alleles has led us to analyze the different RhCE variants investigated by molecular biology. Alloimmunization was analyzed regarding the RHCE genetic profile.
  
  
  METHODS
  Samples from 806 individuals with altered expression of RhCE antigens and/or producing anti-RhCE in the presence of the corresponding antigen were analyzed.
  
  
  RESULTS
  A total of 572 individuals were shown to express RhCE variants. Variant RHCE*ce alleles and RH haplotypes were identified in 83% of cases, the most frequent ones being the R(N) haplotype, the ceMO allele, the (C)ce(s) haplotype/ce(s) 1006 allele, and the ceAR allele identified in 36, 23, 20, and 17% of the tested samples, respectively. The absence of a high-prevalence Rh antigen was documented in 93 individuals. Partial C and partial e were expressed by 53% of individuals with RhCE variants. Rh antibodies were identified in 127 (20%) of 623 patients. They were found to be alloantibodies in 48 (38%) of these 127 patients. Alloimmunization against a high-prevalence Rh antigen was detected in 25% of cases.
  
  
  CONCLUSIONS
  The challenge in clinical red blood cell (RBC) transfusion of patients with sickle cell disease, notably, would be to provide not only phenotypically matched, but also genetically matched, RBC units regarding RhCE variants.
authors.affiliation: Institut National de la Transfusion Sanguine, CNRGS-INSERM U665, Paris, France. bnpham@ints.fr
authors.forename: Bach-Nga
authors.initials: BN
authors.lastname: Pham
authors.forename: Thierry
authors.initials: T
authors.lastname: Peyrard
authors.forename: Genevieve
authors.initials: G
authors.lastname: Juszczak
authors.forename: Marylise
authors.initials: M
authors.lastname: Beolet
authors.forename: Geneviève
authors.initials: G
authors.lastname: Deram
authors.forename: Stéphanie
authors.initials: S
authors.lastname: Martin-Blanc
authors.forename: Isabelle
authors.initials: I
authors.lastname: Dubeaux
authors.forename: Michèle
authors.initials: M
authors.lastname: Roussel
authors.forename: Sandrine
authors.initials: S
authors.lastname: Kappler-Gratias
authors.forename: Dominique
authors.initials: D
authors.lastname: Gien
authors.forename: Sylvie
authors.initials: S
authors.lastname: Poupel
authors.forename: Philippe
authors.initials: P
authors.lastname: Rouger
authors.forename: Pierre-Yves
authors.initials: PY
authors.lastname: Le Pennec
mesh_terms: D000483:Alleles; D000755:Anemia, Sickle Cell / Q000097:blood* / Q000235:genetics*; D005260:Female; D005602:France; D006239:Haplotypes / Q000235:genetics; D006801:Humans; D008297:Male; D011247:Pregnancy; D012204:Rh-Hr Blood-Group System / Q000235:genetics*; D017422:Sequence Analysis, DNA; D065227:Transfusion Reaction
chemical_list: C066468:RHCE protein, human; D012204:Rh-Hr Blood-Group System