Epidemiological and clinical profile of alloimmunized children with sickle cell disease hospitalized in four hospital departments in Libreville

Sickle cell disease is a hereditary disease of the hemoglobin that provides blood transfusion and is responsible for the occurrence of alloimmunization in children with sickle cell disease. 

Objective: The main objective is to describe the epidemiological and clinical profile of alloimmunized children with sickle cell disease hospitalized in four hospital departments in Libreville. 

Materials and methods: This was a prospective descriptive, analytical study that took place over a period of seven months. We included all children with sickle cell disease, whether or not they were being monitored and hospitalized for acute anemia with a history of blood transfusion. 

Results: During our study period, we included 71 children with sickle cell disease. The average age of the children was 80.2±55.4 months. The medical follow-up of children with sickle cell disease was correct 38% (n=27). The inter-critical hemoglobin level was 7g/dl. The medical history was found on average 3 blood transfusions of packed red blood cells. The blood group O positive was the most common 49.3% (n=). Packed red blood cells were the only labile blood product transfused 100% (n=71). The post-transfusion gain in hemoglobin was 1.48±1.3g/dl. The phenotype when incompatible was linked to a low post-transfusion gain. Splenomegaly and jaundice were the post-transfusion complications found with respectively 62% (n=44) and 36.6% (n=26). The Coombs test was positive 26.8% (n=19). 

Conclusion: The phenomenon of alloimmunization remains unknown and underdiagnosed in children with sickle cell disease. The presence of clinical signs of post-transfusion hemolysis requires specific tests to be performed.