Automated red cell exchange as a life-saving modality after ischemic stroke in double heterozygous sickle cell disease with hemoglobin D Punjab

The HbSD is a double heterozygous variant of sickle cell disease (SCD) presenting with a combination of sickle hemoglobin (HbS) and hemoglobin D Punjab (HbD). The homozygous HbD is associated with mild conditions. In contrast, homozygous sickle cell disease (HbSS) presents with severe forms of SCD, like stroke and acute chest syndrome. Occasionally, HbS in the heterozygous state with HbD presents with severe vaso-occlusive complications. Red cell exchange (RCE) is a category I indication in sickle cell patients to prevent and treat stroke, and it improves the rheology by reducing the HBS percentage. Herein, we report a 23-year-old male HbSD patient with a basilar artery stroke. The patient’s pre-procedure HbSD was 71.5% and reduced to 29.5% post-RCE procedure. The patient was on mechanical ventilation at the time of the procedure and had shown a dramatic response with 98% SPO2 on room air after the RCE.