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

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Record number: 
1227
Adverse Occurrence type: 
MPHO Type: 
Estimated frequency: 
Variant RH alleles found in 31/48 (65%) of sickle cell disease (SCD) patients with Rh antibodies. 42% of the antibodies produced by these patients (13 out of 31 patients) with RH variants (anti-D, anti-e and anti-C) were involved in DHTR or decreased survival of transfused RBCs.
Time to detection: 
N/A
Alerting signals, symptoms, evidence of occurrence: 
Worsened anemia and/or increase in HbS was detected in the affected patients.
Demonstration of imputability or root cause: 
It was verified by the change from the patint's baseline pretransfusion Hb and percentage of HbS at the time of antibody detection. The antibodies were associated with worsened anemia and/or an increase in HbS.
Imputability grade: 
3 Definite/Certain/Proven
Groups audience: 
Reference attachment: 
Suggest references: 
Sippert, E., Fujita, C.R., Machado, D., Guelsin, G., Gaspardi, A.C., Pellegrino Jr, J., Gilli, S., Saad, S.S. and Castilho, L. (2014).Variant RH alleles and Rh immunisation in patients with sickle cell disease. Blood Transfus 13(1):72-77.
Note: 
The correct title of this articles is: Variant RH alleles and Rh immunisation in patients with sickle cell disease
Expert comments for publication: 
This article is interesting because provides evidence about how the antibodies identified in patients with RHD variants can be clinically significant being involved in DHTRs. The RH variants characterised have already been associated with Rh alloimmunisation in SCD patients, but in this study only patients with Rh antibodies are included. Knowledge of the prevalence of RH variants and reports such as this one on the clinical significance of antibodies produced by individuals carrying such variants support the development of strategies to match RH to avoid Rh alloimmunisation and the risk of transfusion reactions and/or poor transfusion outcomes. Please consult record #1768 for a discussion of patient genotyping for therapeutic decision making in the context of a delayed serologic transfusion reaction.