Measuring survival of transfused red blood cells in patients with Sickle cell disease and β-Thalassemia using a biotin label approach
β-thalassemia and sickle cell disease (SCD) are hereditary red blood cell (RBC) disorders caused by polymorphisms in the haemoglobin gene, affecting millions of people worldwide. Part of the thalassemia and SCD patients rely on chronic RBC transfusions, aiming at reduction of severe anaemia and prevention of crisis. The transfusion interval differs greatly between patients, ranging from 3 to 8 weeks. This observed variation in transfusion intervals could potentially be explained by differences in RBC survival kinetics.
Little is known about the kinetics of transfused RBCs in patients with SCD and β-thalassemia. It can be hypothesized that the transfused RBCs get destroyed at faster rate as an innocent bystander of a more active immune system. On the contrary, it can be reasoned that the RBCs will persist longer in circulation in those patients as they have a decreased RBC destruction capacity caused by functional asplenia or splenectomy.
We measured survival of one ‘standard matched’ and one ‘genotyped matched’ RBC unit in 9 SCD and 5 β-thalassemia patients using a biotin label approach. Measuring survival of two biotin labeled transfusion units simultaneously in one patient will provide insights into whether patient or donor characteristics determine RBC kinetics and allows for the exploration of the effect of extended blood group matching on RBC clearance rate.
In addition, characteristics of the transfused RBCs over time were measured using flowcytometry (FCM). All samples (6 time points/patient) plus the transfusion mixes were phenotyped using 11 multi-color panels. Understanding (of factors that influence) the kinetics of transfused RBC clearance could facilitate development of safer and more effective (personal) transfusion practices/intervals.