Real-world assessment of acute red cell exchange for stroke in sickle cell disease.

Abstract

Cerebrovascular accidents (CVA) are one of the most devastating complications in sickle cell disease (SCD). Chronic transfusion therapy has been established for primary and secondary prevention of CVA in SCD, resulting in a notable reduction in CVA incidence. For individuals with SCD presenting with CVA, the impact of acute management on neurological outcomes is not well studied. Herein, we examine the neurological outcomes of 29 children and adults with SCD who received acute red cell exchange (RCE) as primary therapy for neurological events at a single institution. Twelve (41%) individuals had a prior history of CVA, and 12 (41%) were on chronic transfusions. Among 13 adults, 3 (23%) had a history of diabetes and 4 (31%) had a history of hypertension. One adult (7.7%) received thrombolytic therapy; the remainder were ineligible due to age, timing of presentation, intracranial haemorrhage or transient symptoms. Higher post-RCE haematocrit was associated with decreased odds of death or persistent neurological symptoms at hospital discharge (OR 0.69, 95% CI: 0.45-0.94, p = 0.017). Optimal acute management of adults living with SCD presenting with CVA remains an understudied yet important topic. Future prospective studies may determine how best to tailor acute management to improve neurological outcomes.