02809nas a2200253   4500008004100000020001400041024001400055245008100069210006900150260027300219300001100492490000700503520180700510100002002317700002302337700002002360700002102380700002102401700002302422700001802445700002202463700002102485856004902506       2015                        eng d  a1723-2007  a10123747900aVariant RH alleles and Rh immunisation in patients with sickle cell disease.0 aVariant RH alleles and Rh immunisation in patients with sickle c  aItalybSippert,Emilia. National Institute of Science and Blood Technology, Campinas Centre Blood, UNICAMP, Campinas, Brazil.Fujita,Claudia R. Fujisan, Fortaleza, Brazil.Machado,Debora. National Institute of Science and Blood Technology, Campinas Centre Blood, UNc2015  a72 - 70 v133 a<p>BACKGROUND: Alloimmunisation is a major complication in patients with sickle cell disease (SCD) receiving red blood cell (RBC) transfusions and despite provision of Rh phenotyped RBC units, Rh antibodies still occur. These antibodies in patients positive for the corresponding Rh antigen are considered autoantibodies in many cases but variant RH alleles found in SCD patients can also contribute to Rh alloimmunisation. In this study, we characterised variant RH alleles in 31 SCD patients who made antibodies to Rh antigens despite antigen-positive status and evaluated the clinical significance of the antibodies produced., MATERIALS AND METHODS: RHD and RHCE BeadChipTM from BioArray Solutions and/or amplification and sequencing of exons were used to identify the RH variants. The serological features of all Rh antibodies in antigen-positive patients were analysed and the clinical significance of the antibodies was evaluated by retrospective analysis of the haemoglobin (Hb) levels before and after transfusion; the change from baseline pre-transfusion Hb and the percentage of HbS were also determined., RESULTS: We identified variant RH alleles in 31/48 (65%) of SCD patients with Rh antibodies. Molecular analyses revealed the presence of partial RHD alleles and variant RHCE alleles associated with altered C and e antigens. Five patients were compound heterozygotes for RHD and RHCE variants. Retrospective analysis showed that 42% of antibodies produced by the patients with RH variants were involved in delayed haemolytic transfusion reactions or decreased survival of transfused RBC., DISCUSSION: In this study, we found that Rh antibodies in SCD patients with RH variants can be clinically significant and, therefore, matching patients based on RH variants should be considered.</p>1 aSippert, Emilia1 aFujita, Claudia, R1 aMachado, Debora1 aGuelsin, Glaucia1 aGaspardi, Ane, C1 aPellegrino, Jordao1 aGilli, Simone1 aSaad, Sara, S T O1 aCastilho, Lilian  uhttps://www.notifylibrary.org/reference/4142