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Diversity of RH and transfusion support in Brazilian sickle cell disease patients with unexplained Rh antibodies.

  • Dinardo, Carla L1, 2
  • Kelly, Shannon3, 4
  • Dezan, Marcia R1
  • Ribeiro, Ingrid H1
  • Castilho, Shirley L5
  • Schimidt, Luciana C6
  • Valgueiro, Maria do C7
  • Preiss, Liliana R8
  • Custer, Brian3
  • Sabino, Ester C2
  • Westhoff, Connie M9
  • 1 Fundação Pró-Sangue Hemocentro de São Paulo, São Paulo, Brazil. , (Brazil)
  • 2 Instituto de Medicina Tropical, Universidade de São Paulo, São Paulo, Brazil. , (Brazil)
  • 3 Vitalant Research Institute, San Francisco, California.
  • 4 UCSF Benioff Children's Hospital Oakland, Oakland, California.
  • 5 Fundação HEMORIO, Rio de Janeiro, Brazil. , (Brazil)
  • 6 Fundação HEMOMINAS, Belo Horizonte, Brazil. , (Brazil)
  • 7 Fundação HEMOPE, Recife, Brazil. , (Brazil)
  • 8 RTI-Research Triangle Institute International, Triangle Park, North Carolina.
  • 9 Laboratory of Immunohematology and Genomics, New York Blood Center, New York, New York.
Published Article
Publication Date
Oct 01, 2019
DOI: 10.1111/trf.15479
PMID: 31408202


Genetic diversity in the RH genes among sickle cell disease (SCD) patients is well described but not yet extensively explored in populations of racially diverse origin. Transfusion support is complicated in patients who develop unexpected Rh antibodies. Our goal was to describe RH variation in a large cohort of Brazilian SCD patients exhibiting unexpected Rh antibodies (antibodies against RH antigens to which the patient is phenotypically positive) and to evaluate the impact of using the patient's RH genotype to guide transfusion support. Patients within the Recipient Epidemiology and Evaluation Donor Study (REDS)-III Brazil SCD cohort with unexpected Rh antibodies were selected for study. RHD and RHCE exons and flanking introns were sequenced by targeted next-generation sequencing. Fifty-four patients with 64 unexplained Rh antibodies were studied. The majority could not be definitively classified as auto- or alloantibodies using serologic methods. The most common altered RH were RHD*DIIIa and RHD*DAR (RHD locus) and RHCE*ce48C, RHCE*ce733G, and RHCE*ceS (RHCE locus). In 53.1% of the cases (34/64), patients demonstrated only conventional alleles encoding the target antigen: five of 12 anti-D (41.7%), 10 of 12 anti-C (83.3%), 18 of 38 anti-e (47.4%), and one of one anti-E (100%). RHD variation in this SCD cohort differs from that reported for African Americans, with increased prevalence of RHD*DAR and underrepresentation of the DAU cluster. Many unexplained Rh antibodies were found in patients with conventional RH allele(s) only. RH genotyping was useful to guide transfusion to determine which patients could potentially benefit from receiving RH genotyped donor units. © 2019 AABB.

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