Systematic Review of Extracorporeal Membrane Oxygenation in Adult Sickle Cell Disease

Background: Sickle cell disease (SCD) is a hereditary hemoglobinopathy associated with life-threatening complications such as acute chest syndrome (ACS), which may necessitate extracorporeal membrane oxygenation (ECMO) in refractory cases. Despite growing use, ECMO in SCD remains challenging due to risks of hemolysis, thrombosis, and anticoagulation complications. This systematic review consolidates existing evidence on ECMO outcomes in SCD, focusing on indications, complications, and survival. Methods: A systematic search of MEDLINE, Cochrane CENTRAL, and Google Scholar was conducted up to January 2025, identifying case reports/series on ECMO use in SCD. Studies reporting venovenous (VV) or venoarterial (VA) ECMO for acute cardiopulmonary failure were included. Data on demographics, laboratory findings, management, and outcomes were extracted. Quality assessment was performed using the Joanna Briggs Institute checklist. Results: Sixteen case reports (23 patients) were included. Most patients were female (65.2%), with ACS (47.8%) and pulmonary embolism (13.0%) as common ECMO indications. VV-ECMO (69.6% of cases) was primarily used for respiratory failure, with a 69% survival rate, while VA-ECMO (30.4%) had a 29% survival rate, often due to cardiogenic shock or cardiac arrest. Complications included hemorrhage (26.1%), neurological injury (21.7%), and thrombosis (13.0%). Exchange transfusion was frequently employed (43.5%), with post-ECMO echocardiography showing improved right ventricular function in survivors. Conclusions: VV-ECMO demonstrates favorable outcomes in SCD-related respiratory failure, whereas VA-ECMO carries higher mortality risks. Careful patient selection, anticoagulation management, and multidisciplinary coordination are essential. Larger prospective studies are needed to refine ECMO utilization in this high-risk population.