Erythrocytapheresis in children with sickle cell disease and acute chest syndrome

Background. Red cell exchange (RCE) is part of the management of acute chest syndrome (ACS) in patients with sickle cell disease. The study describes the indications and outcome of the procedure. Procedure. We retrospectively reviewed 53 episodes of ACS in 44 patients who received RCE from January 2003 to October 2006. Patients were aged between 18 months and 19 years. Results. Sixty-two percent of the patients had at least one previous episode of ACS. ACS was diagnosed at presentation in 66%, in the remainder ACS was diagnosed after a median of 2 days. Clinical Respiratory Score (CRS) was assigned retrospectively to assess respiratory distress (0=no distress, >6=severe). Median admission CRS of 2, progressed to 4 before RCE and declined to 2 within 24 hr afterwards. Median day of RCE was day 2 (IQR 1-3) and the main indication was worsening respiratory distress. No patient developed venous thrombosis, alloantibodies or other complications from RCE. Median length of hospitalization was 7 days (IQR: 5-9 days). Patients with a platelet count significantly lower than their baseline on admission had an increased risk of mechanical ventilation. Conclusions. RCE appears to be a safe and effective treatment for patients with sickle cell disease and ACS. One-third of patients who received RCE for ACS had no respiratory symptoms on admission.