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(Reference retrieved automatically from Web of Science through information on FAPESP grant and its corresponding number as mentioned in the publication by the authors.)

Variant RH alleles and Rh immunisation in patients with sickle cell disease

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Author(s):
Sippert, Emilia [1] ; Fujita, Claudia R. [2] ; Machado, Debora [1] ; Guelsin, Glaucia [1] ; Gaspardi, Ane C. [1] ; Pellegrino, Jr., Jordao [1] ; Gilli, Simone [1] ; Saad, Sara S. T. O. [1] ; Castilho, Lilian [1]
Total Authors: 9
Affiliation:
[1] Univ Estadual Campinas, Natl Inst Sci & Blood Technol, Campinas Ctr Blood, BR-13081970 Campinas, SP - Brazil
[2] Fujisan, Fortaleza, Ceara - Brazil
Total Affiliations: 2
Document type: Journal article
Source: BLOOD TRANSFUSION; v. 13, n. 1, p. 72-77, JAN 2015.
Web of Science Citations: 21
Abstract

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. RI-ID and RHCE BeadChip (TM) 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 RI-ID alleles and variant RHCE alleles associated with altered C and e antigens. Five patients were compound heterozygotes for RI-ID 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. (AU)

FAPESP's process: 13/01426-1 - Immune response to blood antigens in patients with sickle cell disease
Grantee:Emilia Angela Sippert
Support type: Scholarships in Brazil - Doctorate
FAPESP's process: 12/04651-3 - Transfusion safety and immune response to red blood cell antigens in patients with sickle cell disease
Grantee:Lilian Maria de Castilho
Support type: Regular Research Grants