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

Autor(es):Mostrar menos -

Dinardo, Carla L. ^{[1, 2]} ; Kelly, Shannon ^{[3, 4]} ; Dezan, Marcia R. ^[2] ; Ribeiro, Ingrid H. ^[2] ; Castilho, Shirley L. ^[5] ; Schimidt, Luciana C. ^[6] ; Valgueiro, Maria do C. ^[7] ; Preiss, Liliana R. ^[8] ; Custer, Brian ^[3] ; Sabino, Ester C. ^[1] ; Westhoff, Connie M. ^[9] ; Epidemiology, NHLBI Recipient

Afiliação do(s) autor(es)

^[1] Univ Sao Paulo, Inst Med Trop, Sao Paulo - Brazil

^[2] Fundacao Prosangue Hemoctr Sao Paulo, Ave Dr Eneas de Carvalho Aguiar 155, BR-05403000 Sao Paulo - Brazil

^[3] Vitalant Res Inst, San Francisco, CA - USA

^[4] UCSF, Benioff Childrens Hosp Oakland, Oakland, CA - USA

^[5] Fundacao Hemorio, Rio De Janeiro - Brazil

^[6] Fundacao HEMOMINAS, Belo Horizonte, MG - Brazil

^[7] Fundacao HEMOPE, Recife, PE - Brazil

^[8] RTI Res Triangle Inst Int, Res Triangle Pk, NC - USA

^[9] New York Blood Ctr, Lab Immunohematol & Genom, New York, NY 10021 - USA

• Tipo de documento: Artigo Científico
• Fonte: Transfusion; v. 59, n. 10 AUG 2019.
• Citações Web of Science: 0

Resumo

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. (AU)

• Processo FAPESP: 14/50250-6 - Padronização de estratégia molecular custo-efetiva para rastreamento de fenótipos eritrocitários e plaquetários raros em doadores de sangue visando a organização de banco de doadores raros no Estado de SP
• Beneficiário: Ester Cerdeira Sabino
• Modalidade de apoio: Auxílio à Pesquisa - Pesquisa em Políticas Públicas para o SUS