title: High prevalence of red blood cell alloimmunization in sickle cell disease despite transfusion from Rh-matched minority donors.
abstract: Red blood cell (RBC) transfusion is a key treatment of patients with sickle cell disease (SCD) but remains complicated by RBC immunization. In the present study, we evaluated the effects of antigen matching for Rh D, C, and E, and K and transfusion from African American donors in 182 patients with SCD. Overall, 71 (58%) chronic and 9 (15%) episodically transfused patients were alloimmunized. Fifty-five (45%) chronic and 7 (12%) episodically transfused patients were Rh immunized. Of 146 antibodies identified, 91 were unexplained Rh antibodies, one-third of which were associated with laboratory evidence of delayed transfusion reactions. Fifty-six antibodies occurred in patients whose RBCs were phenotypically positive for the corresponding Rh antigen and 35 in patients whose RBCs lacked the antigen and were transfused with Rh-matched RBCs. High-resolution RH genotyping revealed variant alleles in 87% of individuals. These data describe the prevalence of Rh alloimmunization in patients with SCD transfused with phenotypic Rh-matched African American RBCs. Our results suggest that altered RH alleles in both the patients and in the donors contributed to Rh alloimmunization in this study. Whether RH genotyping of patients and minority donors will reduce Rh alloimmunization in SCD needs to be examined.
authors.affiliation: Division of Hematology, The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA. chous@email.chop.edu
authors.forename: Stella T
authors.initials: ST
authors.lastname: Chou
authors.forename: Tannoa
authors.initials: T
authors.lastname: Jackson
authors.forename: Sunitha
authors.initials: S
authors.lastname: Vege
authors.forename: Kim
authors.initials: K
authors.lastname: Smith-Whitley
authors.forename: David F
authors.initials: DF
authors.lastname: Friedman
authors.forename: Connie M
authors.initials: CM
authors.lastname: Westhoff
mesh_terms: D000293:Adolescent; D000328:Adult; D001741:Black or African American; D000755:Anemia, Sickle Cell / Q000235:genetics / Q000276:immunology / Q000628:therapy*; D001789:Blood Group Antigens / Q000276:immunology; D001787:Blood Group Incompatibility / Q000276:immunology / Q000517:prevention & control; D001788:Blood Grouping and Crossmatching; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D017707:Erythrocyte Transfusion; D004912:Erythrocytes / Q000166:cytology*; D005260:Female; D014644:Genetic Variation; D005838:Genotype; D006801:Humans; D007223:Infant; D007518:Isoantibodies / Q000235:genetics* / Q000276:immunology; D008297:Male; D010641:Phenotype; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D001789:Blood Group Antigens; D007518:Isoantibodies; C033242:RH-antibodies