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

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dc.contributorSistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSP
dc.contributor.authorDINARDO, Carla L.
dc.contributor.authorKELLY, Shannon
dc.contributor.authorDEZAN, Marcia R.
dc.contributor.authorRIBEIRO, Ingrid H.
dc.contributor.authorCASTILHO, Shirley L.
dc.contributor.authorSCHIMIDT, Luciana C.
dc.contributor.authorVALGUEIRO, Maria do C.
dc.contributor.authorPREISS, Liliana R.
dc.contributor.authorCUSTER, Brian
dc.contributor.authorSABINO, Ester C.
dc.contributor.authorWESTHOFF, Connie M.
dc.contributor.groupauthorNHLBI Recipient Epidemiology
dc.date.accessioned2019-11-06T18:47:41Z
dc.date.available2019-11-06T18:47:41Z
dc.date.issued2019
dc.description.abstractBACKGROUND 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.eng
dc.description.indexMEDLINEeng
dc.description.sponsorshipSao Paulo Research Foundation (FAPESP)Fundacao de Amparo a Pesquisa do Estado de Sao Paulo (FAPESP) [2014/50250-6]
dc.description.sponsorshipNHLBI Recipient and Evaluation Donor Study (REDS)-III
dc.description.sponsorshipDoris Duke Innovations in Clinical Research Award [2015133]
dc.identifier.citationTRANSFUSION, v.59, n.10, p.3228-3235, 2019
dc.identifier.doi10.1111/trf.15479
dc.identifier.eissn1537-2995
dc.identifier.issn0041-1132
dc.identifier.urihttps://observatorio.fm.usp.br/handle/OPI/34030
dc.language.isoeng
dc.publisherWILEYeng
dc.relation.ispartofTransfusion
dc.rightsrestrictedAccesseng
dc.rights.holderCopyright WILEYeng
dc.subject.otheralloimmunizationeng
dc.subject.otherbloodeng
dc.subject.otheralleleseng
dc.subject.othervariantseng
dc.subject.othertherapyeng
dc.subject.otheranemiaeng
dc.subject.otherriskeng
dc.subject.wosHematologyeng
dc.titleDiversity of RH and transfusion support in Brazilian sickle cell disease patients with unexplained Rh antibodieseng
dc.typearticleeng
dc.type.categoryoriginal articleeng
dc.type.versionpublishedVersioneng
dspace.entity.typePublication
hcfmusp.affiliation.countryEstados Unidos
hcfmusp.affiliation.countryisous
hcfmusp.author.externalKELLY, Shannon:Vitalant Res Inst, San Francisco, CA USA; UCSF, Benioff Childrens Hosp Oakland, Oakland, CA USA
hcfmusp.author.externalCASTILHO, Shirley L.:Fundacao HEMORIO, Rio De Janeiro, Brazil
hcfmusp.author.externalSCHIMIDT, Luciana C.:Fundacao HEMOMINAS, Belo Horizonte, MG, Brazil
hcfmusp.author.externalVALGUEIRO, Maria do C.:Fundacao HEMOPE, Recife, PE, Brazil
hcfmusp.author.externalPREISS, Liliana R.:RTI Res Triangle Inst Int, Res Triangle Pk, NC USA
hcfmusp.author.externalCUSTER, Brian:Vitalant Res Inst, San Francisco, CA USA
hcfmusp.author.externalWESTHOFF, Connie M.:New York Blood Ctr, Lab Immunohematol & Genom, New York, NY 10021 USA
hcfmusp.citation.scopus16
hcfmusp.contributor.author-fmusphcCARLA LUANA DINARDO
hcfmusp.contributor.author-fmusphcMARCIA REGINA DEZAN
hcfmusp.contributor.author-fmusphcINGRID HELENA RIBEIRO
hcfmusp.contributor.author-fmusphcESTER CERDEIRA SABINO
hcfmusp.description.beginpage3228
hcfmusp.description.endpage3235
hcfmusp.description.issue10
hcfmusp.description.volume59
hcfmusp.origemWOS
hcfmusp.origem.pubmed31408202
hcfmusp.origem.scopus2-s2.0-85070687539
hcfmusp.origem.wosWOS:000480806400001
hcfmusp.publisher.cityHOBOKENeng
hcfmusp.publisher.countryUSAeng
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