title: The role of molecular immunohematology in sickle cell disease.
abstract: Red blood cell transfusion therapy is a key component in the treatment of patients with sickle cell disease (SCD). There is no universal standard of care for the appropriate selection of RBC products for patients with SCD. A number of programs extend antigen matching to E and C in the Rh system, and to K, and some attempt to transfuse blood from African-American donors. Although these varied approaches reduce the rate of alloimmunization, patients continue to develop Rh antibodies. Molecular DNA-based analyses of patients alloimmunized to the Rh system, despite serologic Rh antigen matching, invariably reveal altered RH alleles. The prevalence of altered RH alleles in patients with SCD suggests an important emerging role for molecular methods in expanding matching of patients and donors in the Rh system for this patient population.
authors.affiliation: Division of Hematology, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, 3615 Civic Center Boulevard, ARC 316C, Philadelphia, PA 19104, United States. chous@email.chop.edu
authors.forename: Stella T
authors.initials: ST
authors.lastname: Chou
authors.forename: Connie M
authors.initials: CM
authors.lastname: Westhoff
mesh_terms: D001741:Black or African American / Q000235:genetics; D000483:Alleles; D000486:Allergy and Immunology; D000755:Anemia, Sickle Cell / Q000175:diagnosis / Q000235:genetics / Q000628:therapy*; D016477:Artifacts; D001789:Blood Group Antigens / Q000235:genetics / Q000276:immunology; D001787:Blood Group Incompatibility / Q000175:diagnosis / Q000517:prevention & control; D001788:Blood Grouping and Crossmatching / Q000379:methods*; D001803:Blood Transfusion; D004247:DNA / Q000097:blood / Q000235:genetics; D005838:Genotype; D006405:Hematology / Q000379:methods*; D006801:Humans; D007518:Isoantibodies / Q000097:blood / Q000276:immunology; D025202:Molecular Diagnostic Techniques; D012204:Rh-Hr Blood-Group System / Q000235:genetics* / Q000276:immunology
chemical_list: D001789:Blood Group Antigens; D007518:Isoantibodies; D012204:Rh-Hr Blood-Group System; D004247:DNA