RBC alloantibody Rh(e) variant in sickle cell disease

Status: 
Ready to upload
Record number: 
1768
Adverse Occurrence type: 
MPHO Type: 
Estimated frequency: 
Common variant Rh(e) in sickle cell population complicating long term transfusion therapy
Time to detection: 
Delayed serologic. Patient received 8 months of transfusion support prior to developing this "e-like" presentation.
Alerting signals, symptoms, evidence of occurrence: 
Delayed serologic. After 8 leukocytereduced, Rh- and K-matched RBC transfusions from different donors, he developed a complicated alloantibody with “e-like” specificity. The antibody did not react like a simple alloanti-e. It did not represent an autoanti-e either because the patient’s own RBCs were cross-match–compatible with the patient’s antibody in the plasma.
Demonstration of imputability or root cause: 
Genotyping confirms Rh e variant
Imputability grade: 
3 Definite/Certain/Proven
Groups audience: 
Suggest new keywords: 
sickle cell anemia, Rh variant, hematopoietic transplant
Suggest references: 
http://www.ncbi.nlm.nih.gov/pubmed/?term=RH+genotyping+in+a+sickle+cell+disease+patient+contributing+to+hematopoietic+stem+cell+transplantation+donor+selection+and+management
Note: 
Rh variant genotyping helps inform decision to proceed with hematopoietic transplantation.
Expert comments for publication: 
Genotyping helps inform decision to proceed with hematopoietic transplantation as long term transfusion support, given alloimmunization of this patient, may not be possible. Please consult record #1227 for a more general discussion of RBC alloantibodies.