Abstract

Patients with sickle cell disease often undergo frequent blood transfusions. This increases their exposure to red blood cell alloantigens of donor units, thus making it more likely that they produce alloantibodies. This cross-sectional study aimed to describe the prevalence of allo-immunization in patients with sickle cell disease who were monitored at Cayenne Hospital in 2016. Of the 451 patients recruited during the study period, 238 (52.8%) were female. There were 262 (58.1%) homozygous sickle cell and 151 (33.5%) compound heterozygous sickle cell patients. The median age of the participants was 23.09 years (range, 0.5–68). We noted different red blood cell extended phenotypes: -in the Duffy system, the Fya- Fyb–profile was found in 299 patients (66%);—for the Kidd system, the most represented profile was Jka+ Jkb-, with 213 patients (47%). The Jka antigen was present in 355 patients;—in the MNS system, the S-s+ profile was found in 297 patients (66%);—the Lea antigen of the Lewis system was absent in 319 patients. The most frequent Rh phenotype in our patients was D+ C- E- c+ e+ K-, representing 51% of the patients. A total of 6,834 transfused packed red blood cell units were recorded. Sixty-eight patients (23%; 95% confidence interval, 20–25%) had detectable RBC alloantibodies. In multivariate logistic regression, only the mean number of single transfusions was statistically higher for the alloimmunized patients (p < 0.04). Thirteen (19%) of the patients with alloimmunization developed a delayed hemolytic transfusion reaction, thus representing 4.4% of the total number of transfused patients. Whether differences between donors from France vs. recipients from French Guiana could explain this high prevalence of alloimmunization to be examined. In conclusion, careful transfusion strategies for patients with RBC alloantibodies should allow further reduction of the rate of alloimmunization.

Highlights

  • Red blood cell (RBC) transfusions are often used to treat acute complications of sickle cell disease (SCD)

  • This study aimed to describe the prevalence of alloimmunization in patients with SCD, since the transition away from blood donation in French Guiana

  • This was a cross-sectional study designed to describe the following immunohematological characteristics of sickle cell patients followed in our center: the number of transfusion episodes, number of units of transfused blood, indication for transfusion, age at first transfusion, history of pregnancy if necessary, blood group ABO, D and the extended phenotypes, the possible type of alloantibodies, the rate of alloimmunization, and types of red blood cell alloantibodies in patients with SCD who were being monitored at the sickle cell center of Cayenne Hospital in 2016

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Summary

Introduction

Red blood cell (RBC) transfusions are often used to treat acute complications of sickle cell disease (SCD). Many patients with SCD, undergo numerous transfusions over the course of their lives, thereby greatly increasing their exposure to RBC alloantigens of donor units. These patients are, more likely to produce alloantibodies. The packed RBC units are transported by air to the Guianese French Blood Establishment The latter facility only ensures their delivery to the hospitals where the transfusion is to be provided. This study aimed to describe the prevalence of alloimmunization in patients with SCD, since the transition away from blood donation in French Guiana

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