Reconsidering sickle cell trait testing of red blood cell units allocated to children with sickle cell disease.

ABSTRACT

BACKGROUND: Sickle cell trait (SCT) testing of red blood cell (RBC) units is sometimes performed to identify and divert units containing hemoglobin S (HbS). Recipients strategically guarded against this exposure include fetuses, neonates, and children with sickle cell disease (SCD). The clinical necessity of this practice is unclear. STUDY DESIGN AND METHODS: A one-year audit (2018) was performed at a pediatric tertiary care hospital that tests for SCT in RBC units prescribed to children with SCD and neonates. The impact of incorporating varying numbers of SCT RBC units in a single-unit top-up, partial-manual red cell exchange, and automated erythrocytapheresis was modeled in four typical-parameter age scenarios (2, 5, 10, and 18 years) sharing a high baseline HbS. Additionally, a survey assessing SCT testing practices was administered to Canadian pediatric hospital transfusion laboratories serving hemoglobinopathy programs. RESULTS: Of 2268 donor RBC units tested, one was positive for SCT (0.04% [95% CI 0.01%-0.24%]), at a cost of $19,384.56 CAD. The impact of SCT unit incorporation on lost HbS reduction was modest (Δ1%-3% [automated erythrocytapheresis] and Δ4%-15% [top-up/partial manual exchange]). The survey (with all 13 sites responding) showed variable SCT testing practice; four (31%) do not test, four (31%) test for children with SCD, and six (46%) test for neonates. CONCLUSION: RBC SCT testing may be more costly than beneficial or necessary in children with SCD. As of 2019, our transfusion service has ceased SCT testing for this population. Further research in the fetal/neonatal populations is needed to overturn this entrenched practice.