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Diversity of RH and transfusion support in Brazilian sickle cell disease patients with unexplained Rh antibodies.
Dinardo, Carla L; Kelly, Shannon; Dezan, Marcia R; Ribeiro, Ingrid H; Castilho, Shirley L; Schimidt, Luciana C; Valgueiro, Maria do C; Preiss, Liliana R; Custer, Brian; Sabino, Ester C; Westhoff, Connie M.
Afiliação
  • Dinardo CL; Fundação Pró-Sangue Hemocentro de São Paulo, São Paulo, Brazil.
  • Kelly S; Instituto de Medicina Tropical, Universidade de São Paulo, São Paulo, Brazil.
  • Dezan MR; Vitalant Research Institute, San Francisco, California.
  • Ribeiro IH; UCSF Benioff Children's Hospital Oakland, Oakland, California.
  • Castilho SL; Fundação Pró-Sangue Hemocentro de São Paulo, São Paulo, Brazil.
  • Schimidt LC; Fundação Pró-Sangue Hemocentro de São Paulo, São Paulo, Brazil.
  • Valgueiro MDC; Fundação HEMORIO, Rio de Janeiro, Brazil.
  • Preiss LR; Fundação HEMOMINAS, Belo Horizonte, Brazil.
  • Custer B; Fundação HEMOPE, Recife, Brazil.
  • Sabino EC; RTI-Research Triangle Institute International, Triangle Park, North Carolina.
  • Westhoff CM; Vitalant Research Institute, San Francisco, California.
Transfusion ; 59(10): 3228-3235, 2019 10.
Article em En | MEDLINE | ID: mdl-31408202
BACKGROUND: 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. STUDY DESIGN AND METHODS: 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. RESULTS: 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%). CONCLUSION: 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.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Sistema do Grupo Sanguíneo Rh-Hr / Transfusão de Sangue / Alelos / Genótipo / Isoanticorpos Tipo de estudo: Clinical_trials / Evaluation_studies / Risk_factors_studies Limite: Female / Humans / Male País/Região como assunto: America do sul / Brasil Idioma: En Revista: Transfusion Ano de publicação: 2019 Tipo de documento: Article País de afiliação: Brasil País de publicação: Estados Unidos
Texto completo
Adicionar na Minha BVS
Imprimir
XML
PubMed Links
Buscar no Google

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Sistema do Grupo Sanguíneo Rh-Hr / Transfusão de Sangue / Alelos / Genótipo / Isoanticorpos Tipo de estudo: Clinical_trials / Evaluation_studies / Risk_factors_studies Limite: Female / Humans / Male País/Região como assunto: America do sul / Brasil Idioma: En Revista: Transfusion Ano de publicação: 2019 Tipo de documento: Article País de afiliação: Brasil País de publicação: Estados Unidos