Impact of red blood cell alloimmunization on sickle cell disease mortality: a case series.
BACKGROUND
Although red blood cell (RBC) transfusion represents an integral component of sickle cell disease (SCD) care, transfusion support for some patients can result in alloimmunization to RBC antigens. Alloimmunized patients with SCD appear to experience worse survival compared to nonalloimmunized patients. While this difference in mortality may in part be due to underlying immunologic differences related to disease severity, it may also reflect direct clinical consequences of RBC alloimmunization. Alloimmunized patients have an increased risk of serious hemolytic transfusion reactions (HTRs) and may not receive adequate RBC transfusion support due to lack of compatible RBC units.


METHODS
This study reports on five RBC alloimmunized patients with SCD who died, to illustrate the concept that RBC alloimmunization itself contributes to premature death.


RESULTS
The clinical course for each of the reported patients provides insight into the direct and indirect consequences of RBC alloimmunization, where patients experienced delayed HTRs or did not receive needed RBC transfusions.


CONCLUSIONS
Future work examining the clinical impact of RBC alloimmunization should not only consider HTRs but should also address the potential consequences associated with difficulties in obtaining compatible blood.
Center for Transfusion and Cellular Therapy, Department of Pathology, Emory University, Atlanta, Georgia.|Division of Hematology, Children's National Health System, Washington, DC.
Robert Sheppard
RS
Nickel
Center for Transfusion and Cellular Therapy, Department of Pathology, Emory University, Atlanta, Georgia.|Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta, Emory University, Atlanta, Georgia.|Department of Pediatrics, Yale University, New Haven, Connecticut.|Department of Laboratory Medicine, Yale University, New Haven, Connecticut.
Jeanne E
JE
Hendrickson
Center for Transfusion and Cellular Therapy, Department of Pathology, Emory University, Atlanta, Georgia.|Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta, Emory University, Atlanta, Georgia.
Ross M
RM
Fasano
Center for Transfusion and Cellular Therapy, Department of Pathology, Emory University, Atlanta, Georgia.
Erin K
EK
Meyer
Center for Transfusion and Cellular Therapy, Department of Pathology, Emory University, Atlanta, Georgia.
Anne M
AM
Winkler
Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta, Emory University, Atlanta, Georgia.
Marianne M
MM
Yee
Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta, Emory University, Atlanta, Georgia.
Peter A
PA
Lane
Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta, Emory University, Atlanta, Georgia.
Yuritzi A
YA
Jones
Department of Pediatrics, Yale University, New Haven, Connecticut.
Farzana D
FD
Pashankar
Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta, Emory University, Atlanta, Georgia.
Tamara
T
New
Center for Transfusion and Cellular Therapy, Department of Pathology, Emory University, Atlanta, Georgia.|Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta, Emory University, Atlanta, Georgia.
Cassandra D
CD
Josephson
Center for Transfusion and Cellular Therapy, Department of Pathology, Emory University, Atlanta, Georgia.
Sean R
SR
Stowell
D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood / Q000276:immunology / Q000401:mortality* / Q000628:therapy; D015415:Biomarkers / Q000097:blood; D017707:Erythrocyte Transfusion / Q000009:adverse effects*; D004912:Erythrocytes / Q000276:immunology*; D005260:Female; D006801:Humans; D007518:Isoantibodies / Q000097:blood*; D008297:Male; D065227:Transfusion Reaction / Q000097:blood / Q000175:diagnosis / Q000276:immunology / Q000401:mortality*; D055815:Young Adult
D015415:Biomarkers; D007518:Isoantibodies