Alloimmunization and Age of Death among Deceased Sickle Cell Disease Patients Open Access

Nielsen, Kyron (2016)

Permanent URL: https://etd.library.emory.edu/concern/etds/1r66j129x?locale=en
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Abstract

Transfusion therapy remains a primary treatment for Sickle Cell Disease (SCD). Alloimmunization frequently occurs as a result of the incompatibility of donor and recipient blood types. The association between alloimmunization and morbidity and mortality is not completely understood. Some studies have suggested an association between alloimmunization and increased morbidities as well as decreased life expectancy, though research in this area is lacking. We investigated the association between alloimmunization and the age at death among a cohort of 136 patients who died from SCD in Georgia from 2004-2008. Patients were identified from a previous study and were linked to existing medical records in several hospital systems. Variables of interest included birth date, death date, alloimmunization status, antibody profile, phenotype, number of transfused units, type of hemoglobinopathy, cause of death, ABO/Rh blood type, and date of last antibody screen. A red blood cell index was calculated to estimate the number of units needed to crossmatch in order to find a compatible unit based on the patient's phenotype, antibody profile, and the average donor pool. The mean age at death of those who were alloimmunized was 38.5 years, compared to 39.5 years for those who were not alloimmunized (p=0.72). The mean age at death was 40.3 years for those with a low (<20) number of transfusions, compared to 33.4 years for those with a high (20 or more) number of transfusions (p=0.05). Neither the total number of antibodies, nor the presence of specific antibodies affected the age at death among alloimmunized patients. Multivariate analysis showed that decreased age at death was not associated with the red blood cell index, total number of antibodies, or number of transfusions. These findings differ from some earlier studies due to a number of limitations in study design. To further assess the association between alloimmunization and survival, a prospective cohort study is needed.

Table of Contents

Introduction............................................................1

Methods..................................................................6

Results....................................................................9

Discussion..............................................................11

References..............................................................14

Tables and Figures..................................................19

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