Alloimmunization in Egyptian children with sickle cell disease

Abstract

Background Although transfusion is a cornerstone in the treatment of sickle cell disease (SCD), the subsequent erythrocyte alloimmunization creates a significant challenge in their management.Aim The aim was to measure the frequency of the occurrence of the alloimmune markers in SCD and to investigate its predicators.Patients and methods This cross-sectional study recruited 50 children and young adults with SCD. All patients were subjected to detailed medical history and thorough clinical examination. Laboratory investigations included complete blood count, markers of hemolysis, and serum ferritin. The D, C, c, E, e, M, NJKa, and JKb antigens were typed using monoclonal antisera. Fya, Fyb, Jka, Jkb, S, s, and anti-Lua antigens were typed by commercially prepared polyclonal antihuman sera. Antibody screening was performed using ortho screening panel.Results Sickle SS was the most common type of SCD, representing more than half of the recruited patients. The frequency of antibody positive screening among SCD was 16%. Higher frequency of alloimmunization was recorded in those with SB+ than those SS and SB0. Patients with SCD who had alloimmunization were significantly older when they received their first blood transfusion and had lower transfusion index (P=0.037) than those with negative antibodies screening (P=0.013). There were no significant differences between patients with alloimmunization and those without regarding age, sex, transfusion frequency, and SCD comorbidities; painful crisis (P=0.117), stroke (P=0.398), acute chest syndrome (ACS) (P=0.363).Conclusion Alloimmunization in patients with SCD has important clinical significance, especially those who require regular blood transfusion. Consequently early recognition and management of alloimunization is necessary.