Volume 56, Issue 1 pp. 107-114
IMMUNOHEMATOLOGY

Impact of red blood cell alloimmunization on sickle cell disease mortality: a case series

Robert Sheppard Nickel

Robert Sheppard Nickel

Center for Transfusion and Cellular Therapy, Department of Pathology, Emory University, Atlanta, Georgia

Division of Hematology, Children's National Health System, Washington, DC

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Jeanne E. Hendrickson

Jeanne E. 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

Department of Pediatrics, Yale University, New Haven, Connecticut

Department of Laboratory Medicine, Yale University, New Haven, Connecticut

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Ross M. Fasano

Ross M. Fasano

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

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Erin K. Meyer

Erin K. Meyer

Center for Transfusion and Cellular Therapy, Department of Pathology, Emory University, Atlanta, Georgia

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Anne M. Winkler

Anne M. Winkler

Center for Transfusion and Cellular Therapy, Department of Pathology, Emory University, Atlanta, Georgia

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Marianne M. Yee

Marianne M. Yee

Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta, Emory University, Atlanta, Georgia

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Peter A. Lane

Peter A. Lane

Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta, Emory University, Atlanta, Georgia

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Yuritzi A. Jones

Yuritzi A. Jones

Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta, Emory University, Atlanta, Georgia

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Farzana D. Pashankar

Farzana D. Pashankar

Department of Pediatrics, Yale University, New Haven, Connecticut

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Tamara New

Tamara New

Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta, Emory University, Atlanta, Georgia

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Cassandra D. Josephson

Cassandra D. Josephson

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

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Sean R. Stowell

Corresponding Author

Sean R. Stowell

Center for Transfusion and Cellular Therapy, Department of Pathology, Emory University, Atlanta, Georgia

Address reprint requests to: Sean R. Stowell, Center for Transfusion and Cellular Therapy, Department of Pathology, Emory University, Atlanta, GA 30322; e-mail: [email protected]; [email protected].Search for more papers by this author
First published: 28 October 2015
Citations: 98

Abstract

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.

CASE REPORT

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.

CONCLUSION

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.

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