A Case Report of Alloimmunization with Hyperhaemolysis in a Multiple Transfused Known SCD Patient at a Tertiary Hospital South Nigeria: A Challenge in Developing Countries

Abstract

Background: Hyperhaemolysis is a fatal life threatening haemolytic transfusion reaction which is common among sickle cell disease patient. Aim: The aim is to create the awareness of the increasing trend of hyperhaemolysis among chronic transfused sickle cell patients and to educate medical practitioners on the means of ameliorating this menace. Presentation of Case: The patient was a 14 years, old male; known sickle cell anaemia (Hbss) diagnosed 13 years ago with Haemoglobin electrophoresis. Patient’s present condition was said to have worsen 4 years ago when patient crisis became so frequent with about 6-8 episode per year, which is so severe that at each episode patient is said to have received between 2-5 unit of whole blood. Further work up revealed a negative direct antiglobulin test (DAT) but Indirect antiglobulin test (IAT) was positive demonstrating the presence of anti-Jka alloantibodies. Patient RBC were phenotyped and was found to be JKa negative, whereas patient blood group is O+ and had received several blood transfusion in our centre where extended phenotyping is not done for the past 3 decades. Each transfusion precipitated a drop in Hb and Hct to levels lower than before transfusion; once transfusions were withheld, the patient slowly recovered. Summary: Hyperhaemolysis in the setting of alloimmunization is a common complication among multiple transfused SCD which is life threatening requires prompt intervention if taking into consideration among managing physician and also ensure measure to prevent it from occurring. Conclusion: Transfusions worsens features of hyperhaemolysis in SCD patients. Extended red cell phenotyping may reduce associated risk.